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Sample records for syndrome critical region

  1. Constitutional chromothripsis involving the critical region of 9q21.13 microdeletion syndrome.

    Science.gov (United States)

    Genesio, Rita; Fontana, Paolo; Mormile, Angela; Casertano, Alberto; Falco, Mariateresa; Conti, Anna; Franzese, Adriana; Mozzillo, Enza; Nitsch, Lucio; Melis, Daniela

    2015-01-01

    The chromothripsis is a biological phenomenon, first observed in tumors and then rapidly described in congenital disorders. The principle of the chromothripsis process is the occurrence of a local shattering to pieces and rebuilding of chromosomes in a random order. Congenital chromothripsis rearrangements often involve reciprocal rearrangements on multiple chromosomes and have been described as cause of contiguous gene syndromes. We hypothesize that chromothripsis could be responsible for known 9q21.13 microdeletion syndrome, causing a composite phenotype with additional features. The case reported is a 16- years-old female with a complex genomic rearrangement of chromosome 9 including the critical region of 9q21.13 microdeletion syndrome. The patient presents with platelet disorder and thyroid dysfunction in addition to the classical neurobehavioral phenotype of the syndrome. The presence of multiple rearrangements on the same chromosome 9 and the rebuilding of chromosome in a random order suggested that the rearrangement could origin from an event of chromthripsis. To our knowledge this is the first report of congenital chromothripsis involving chromosome 9. Furthermore this is the only case of 9q21.13 microdeletion syndrome due to chromothripsis.

  2. Cytogenomic Integrative Network Analysis of the Critical Region Associated with Wolf-Hirschhorn Syndrome

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    Thiago Corrêa

    2018-01-01

    Full Text Available Deletions in the 4p16.3 region are associated with Wolf-Hirschhorn syndrome (WHS, a contiguous gene deletion syndrome involving variable size deletions. In this study, we perform a cytogenomic integrative analysis combining classical cytogenetic methods, fluorescence in situ hybridization (FISH, chromosomal microarray analysis (CMA, and systems biology strategies, to establish the cytogenomic profile involving the 4p16.3 critical region and suggest WHS-related intracellular cell signaling cascades. The cytogenetic and clinical patient profiles were evaluated. We characterized 12 terminal deletions, one interstitial deletion, two ring chromosomes, and one classical translocation 4;8. CMA allowed delineation of the deletions, which ranged from 3.7 to 25.6 Mb with breakpoints from 4p16.3 to 4p15.33. Furthermore, the smallest region of overlapping (SRO encompassed seven genes in a terminal region of 330 kb in the 4p16.3 region, suggesting a region of susceptibility to convulsions and microcephaly. Therefore, molecular interaction networks and topological analysis were performed to understand these WHS-related symptoms. Our results suggest that specific cell signaling pathways including dopamine receptor, NAD+ nucleosidase activity, and fibroblast growth factor-activated receptor activity are associated with the diverse pathological WHS phenotypes and their symptoms. Additionally, we identified 29 hub-bottlenecks (H-B nodes with a major role in WHS.

  3. Mapping the Wolf-Hirschhorn syndrome phenotype outside the currently accepted WHS critical region and defining a new critical region, WHSCR-2.

    Science.gov (United States)

    Zollino, Marcella; Lecce, Rosetta; Fischetto, Rita; Murdolo, Marina; Faravelli, Francesca; Selicorni, Angelo; Buttè, Cinzia; Memo, Luigi; Capovilla, Giuseppe; Neri, Giovanni

    2003-03-01

    In an attempt to define the distinctive Wolf-Hirschhorn syndrome (WHS) phenotype, and to map its specific clinical manifestations, a total of eight patients carrying a 4p16.3 microdeletion were analyzed for their clinical phenotype and their respective genotypes. The extent of each individual deletion was established by fluorescence in situ hybridization, with a cosmid contig spanning the genomic region from MSX1 (distal half of 4p16.1) to the subtelomeric locus D4S3359. The deletions were 1.9-3.5 Mb, and all were terminal. All the patients presented with a mild phenotype, in which major malformations were usually absent. It is worth noting that head circumference was normal for height in two patients (those with the smallest deletions [1.9 and 2.2 Mb]). The currently accepted WHS critical region (WHSCR) was fully preserved in the patient with the 1.9-Mb deletion, in spite of a typical WHS phenotype. The deletion in this patient spanned the chromosome region from D4S3327 (190 b4 cosmid clone included) to the telomere. From a clinical point of view, the distinctive WHS phenotype is defined by the presence of typical facial appearance, mental retardation, growth delay, congenital hypotonia, and seizures. These signs represent the minimal diagnostic criteria for WHS. This basic phenotype maps distal to the currently accepted WHSCR. Here, we propose a new critical region for WHS, and we refer to this region as "WHSCR-2." It falls within a 300-600-kb interval in 4p16.3, between the loci D4S3327 and D4S98-D4S168. Among the candidate genes already described for WHS, LETM1 (leucine zipper/EF-hand-containing transmembrane) is likely to be pathogenetically involved in seizures. On the basis of genotype-phenotype correlation analysis, dividing the WHS phenotype into two distinct clinical entities, a "classical" and a "mild" form, is recommended for the purpose of proper genetic counseling.

  4. Constitutional and somatic deletions of the Williams-Beuren syndrome critical region in non-Hodgkin lymphoma.

    Science.gov (United States)

    Guenat, David; Quentin, Samuel; Rizzari, Carmelo; Lundin, Catarina; Coliva, Tiziana; Edery, Patrick; Fryssira, Helen; Bermont, Laurent; Ferrand, Christophe; Soulier, Jean; Borg, Christophe; Rohrlich, Pierre-Simon

    2014-11-07

    Here, we report and investigate the genomic alterations of two novel cases of Non-Hodgkin Lymphoma (NHL) in children with Williams-Beuren syndrome (WBS), a multisystem disorder caused by 7q11.23 hemizygous deletion. Additionally, we report the case of a child with NHL and a somatic 7q11.23 deletion. Although the WBS critical region has not yet been identified as a susceptibility locus in NHL, it harbors a number of genes involved in DNA repair. The high proportion of pediatric NHL reported in WBS is intriguing. Therefore, the role of haploinsufficiency of genes located at 7q11.23 in lymphomagenesis deserves to be investigated.

  5. Down syndrome critical region 2 protein inhibits the transcriptional activity of peroxisome proliferator-activated receptor β in HEK293 cells

    International Nuclear Information System (INIS)

    Song, Hae Jin; Park, Joongkyu; Seo, Su Ryeon; Kim, Jongsun; Paik, Seung R.; Chung, Kwang Chul

    2008-01-01

    Down syndrome is mainly caused by a trisomy of chromosome 21. The Down syndrome critical region 2 (DSCR2) gene is located within a part of chromosome 21, the Down syndrome critical region (DSCR). To investigate the function of DSCR2, we sought to identify DSCR2-interacting proteins using yeast two-hybrid assays. A human fetal brain cDNA library was screened, and DSCR2 was found to interact with a member of the nuclear receptor superfamily, peroxisome proliferator-activated receptor β, (PPARβ). A co-immunoprecipitation assay demonstrated that DSCR2 physically interacts with PPARβ in mammalian HEK293 cells. DSCR2 also inhibited the ligand-induced transcriptional activity of PPARβ. Furthermore, PPARβ also decreased the solubility of DSCR2, which increased levels of insoluble DSCR2

  6. VEGF selectively induces Down syndrome critical region 1 gene expression in endothelial cells: a mechanism for feedback regulation of angiogenesis?

    International Nuclear Information System (INIS)

    Yao, Y.-G; Duh, Elia J.

    2004-01-01

    The Down syndrome critical region 1 (DSCR1) gene (also known as MCIP1, Adapt78) encodes a regulatory protein that binds to calcineurin catalytic A subunit and acts as a regulator of the calcineurin-mediated signaling pathway. We show in this study that DSCR1 is greatly induced in endothelial cells in response to VEGF, TNF-α, and A23187 treatment, and that this up-regulation is inhibited by inhibitors of the calcineurin-NFAT (nuclear factor of activated T cells) signaling pathway as well as by PKC inhibition and a Ca 2+ chelator. We hypothesized that the up-regulation of DSCR1 gene expression in endothelial cells could act as an endogenous feedback inhibitor for angiogenesis by regulating the calcineurin-NFAT signaling pathway. Our transient transfection analyses confirm that the overexpression of DSCR1 abrogates the up-regulation of reporter gene expression driven by both the cyclooxygenase 2 and DSCR1 promoters in response to stimulators. Our results indicate that DSCR1 up-regulation may represent a potential molecular mechanism underlying the regulation of angiogenic genes activated by the calcineurin-NFAT signaling pathway in endothelial cells

  7. A Paternally Inherited Duplication in the Prader-Willi/Angelman Syndrome Critical Region: A Case and Family Study

    Science.gov (United States)

    Veltman, Marijcke W. M.; Thompson, Russell J.; Craig, Ellen E.; Dennis, Nicholas R.; Roberts, Sian E.; Moore, Vanessa; Brown, Josie A.; Bolton, Patrick F.

    2005-01-01

    The Prader-Willi/Angelman Critical Region (PWACR; Chromosome 15q11-13) is of interest as a potential locus for genes conferring susceptibility to autism spectrum disorders (ASD). This report describes a female proband referred for evaluation of a possible ASD. Genetic analyses indicated that the proband, her father and one of her sisters, carried…

  8. Complex Regional Pain Syndrome

    Science.gov (United States)

    Complex regional pain syndrome (CRPS) is a chronic pain condition. It causes intense pain, usually in the arms, hands, legs, or feet. It may happen ... move the affected body part The cause of CRPS is unknown. There is no specific diagnostic test. ...

  9. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice

    Science.gov (United States)

    Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D. Woodrow; Ivy, Dunbar; Perryman, M.B.; Garrett-Sinha, Lee Ann; Grossfeld, Paul

    2010-01-01

    Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an ∼7 Mb ‘cardiac critical region’ in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease. PMID:19942620

  10. Epilepsy is a possible feature in Williams-Beuren syndrome patients harboring typical deletions of the 7q11.23 critical region.

    Science.gov (United States)

    Nicita, Francesco; Garone, Giacomo; Spalice, Alberto; Savasta, Salvatore; Striano, Pasquale; Pantaleoni, Chiara; Spartà, Maria Valentina; Kluger, Gerhard; Capovilla, Giuseppe; Pruna, Dario; Freri, Elena; D'Arrigo, Stefano; Verrotti, Alberto

    2016-01-01

    Seizures are rarely reported in Williams-Beuren syndrome (WBS)--a contiguous-gene-deletion disorder caused by a 7q11.23 heterozygous deletion of 1.5-1.8 Mb--and no previous study evaluated electro-clinical features of epilepsy in this syndrome. Furthermore, it has been hypothesized that atypical deletion (e.g., larger than 1.8 Mb) may be responsible for a more pronounced neurological phenotypes, especially including seizures. Our objectives are to describe the electro-clinical features in WBS and to correlate the epileptic phenotype with deletion of the 7q11.23 critical region. We evaluate the electro-clinical features in one case of distal 7q11.23 deletion syndrome and in eight epileptic WBS (eWBS) patients. Additionally, we compare the deletion size-and deleted genes-of four epileptic WBS (eWBS) with that of four non-epileptic WBS (neWBS) patients. Infantile spasms, focal (e.g., motor and dyscognitive with autonomic features) and generalized (e.g., tonic-clonic, tonic, clonic, myoclonic) seizures were encountered. Drug-resistance was observed in one patient. Neuroimaging discovered one case of focal cortical dysplasia, one case of fronto-temporal cortical atrophy and one case of periventricular nodular heterotopia. Comparison of deletion size between eWBS and neWBS patients did not reveal candidate genes potentially underlying epilepsy. This is the largest series describing electro-clinical features of epilepsy in WBS. In WBS, epilepsy should be considered both in case of typical and atypical deletions, which do not involve HIP1, YWHAG or MAGI2. © 2015 Wiley Periodicals, Inc.

  11. Prader-Willi Critical Region, a Non-Translated, Imprinted Central Regulator of Bone Mass: Possible Role in Skeletal Abnormalities in Prader-Willi Syndrome.

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    Ee-Cheng Khor

    Full Text Available Prader-Willi Syndrome (PWS, a maternally imprinted disorder and leading cause of obesity, is characterised by insatiable appetite, poor muscle development, cognitive impairment, endocrine disturbance, short stature and osteoporosis. A number of causative loci have been located within the imprinted Prader-Willi Critical Region (PWCR, including a set of small non-translated nucleolar RNA's (snoRNA. Recently, micro-deletions in humans identified the snoRNA Snord116 as a critical contributor to the development of PWS exhibiting many of the classical symptoms of PWS. Here we show that loss of the PWCR which includes Snord116 in mice leads to a reduced bone mass phenotype, similar to that observed in humans. Consistent with reduced stature in PWS, PWCR KO mice showed delayed skeletal development, with shorter femurs and vertebrae, reduced bone size and mass in both sexes. The reduction in bone mass in PWCR KO mice was associated with deficiencies in cortical bone volume and cortical mineral apposition rate, with no change in cancellous bone. Importantly, while the length difference was corrected in aged mice, consistent with continued growth in rodents, reduced cortical bone formation was still evident, indicating continued osteoblastic suppression by loss of PWCR expression in skeletally mature mice. Interestingly, deletion of this region included deletion of the exclusively brain expressed Snord116 cluster and resulted in an upregulation in expression of both NPY and POMC mRNA in the arcuate nucleus. Importantly, the selective deletion of the PWCR only in NPY expressing neurons replicated the bone phenotype of PWCR KO mice. Taken together, PWCR deletion in mice, and specifically in NPY neurons, recapitulates the short stature and low BMD and aspects of the hormonal imbalance of PWS individuals. Moreover, it demonstrates for the first time, that a region encoding non-translated RNAs, expressed solely within the brain, can regulate bone mass in health

  12. Korsakoff's syndrome: A critical review

    NARCIS (Netherlands)

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

    2017-01-01

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

  13. WHSC1, a 90 kb SET domain-containing gene, expressed in early development and homologous to a Drosophila dysmorphy gene maps in the Wolf-Hirschhorn syndrome critical region and is fused to IgH in t(4;14) multiple myeloma

    NARCIS (Netherlands)

    Stec, I.; Wright, T. J.; van Ommen, G. J.; de Boer, P. A.; van Haeringen, A.; Moorman, A. F.; Altherr, M. R.; den Dunnen, J. T.

    1998-01-01

    Wolf-Hirschhorn syndrome (WHS) is a malformation syndrome associated with a hemizygous deletion of the distal short arm of chromosome 4 (4p16.3). The smallest region of overlap between WHS patients, the WHS critical region, has been confined to 165 kb, of which the complete sequence is known. We

  14. Complex Regional Pain Syndrome and Treatment Approaches

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    Neslihan Gokcen

    2013-08-01

    Full Text Available Complex Regional Pain Syndrome is a symptom complex including severe pain which is disproportioned by the initiating event. Formerly, it was known as reflex sympathetic dystropy, Sudeck’s atrophy and algoneurodystrophy. There are two types of complex regional pain syndrome (CPRS. CRPS type 1 (Reflex sympathetic dystropy occurs after a minor trauma of the extremities, CRPS type 2 (Causalgia occurs following peripheral nevre injury. Diagnosis is made according to the history, symptoms and physical findings of the patients. Patient education, physical therapy and medical treatment are the most common treatment approaches of complex regional pain syndrome. The aim of this review is to revise the treatment options ofcomplex regional pain syndrome, as well as to overview the new treatment approaches and options for the refractory complex regional pain syndrome cases. [Archives Medical Review Journal 2013; 22(4.000: 514-531

  15. The App-Runx1 region is critical for birth defects and electrocardiographic dysfunctions observed in a Down syndrome mouse model.

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    Matthieu Raveau

    2012-05-01

    Full Text Available Down syndrome (DS leads to complex phenotypes and is the main genetic cause of birth defects and heart diseases. The Ts65Dn DS mouse model is trisomic for the distal part of mouse chromosome 16 and displays similar features with post-natal lethality and cardiovascular defects. In order to better understand these defects, we defined electrocardiogram (ECG with a precordial set-up, and we found conduction defects and modifications in wave shape, amplitudes, and durations in Ts65Dn mice. By using a genetic approach consisting of crossing Ts65Dn mice with Ms5Yah mice monosomic for the App-Runx1 genetic interval, we showed that the Ts65Dn viability and ECG were improved by this reduction of gene copy number. Whole-genome expression studies confirmed gene dosage effect in Ts65Dn, Ms5Yah, and Ts65Dn/Ms5Yah hearts and showed an overall perturbation of pathways connected to post-natal lethality (Coq7, Dyrk1a, F5, Gabpa, Hmgn1, Pde10a, Morc3, Slc5a3, and Vwf and heart function (Tfb1m, Adam19, Slc8a1/Ncx1, and Rcan1. In addition cardiac connexins (Cx40, Cx43 and sodium channel sub-units (Scn5a, Scn1b, Scn10a were found down-regulated in Ts65Dn atria with additional down-regulation of Cx40 in Ts65Dn ventricles and were likely contributing to conduction defects. All these data pinpoint new cardiac phenotypes in the Ts65Dn, mimicking aspects of human DS features and pathways altered in the mouse model. In addition they highlight the role of the App-Runx1 interval, including Sod1 and Tiam1, in the induction of post-natal lethality and of the cardiac conduction defects in Ts65Dn. These results might lead to new therapeutic strategies to improve the care of DS people.

  16. Spontaneous onset of Complex Regional Pain Syndrome

    NARCIS (Netherlands)

    de Rooij, A.M.; Perez, R.S.G.M.; Huygen, F.J.; van Eijs, F.; van Kleef, M.; Bauer, M.C.R.; van Hilten, J.J.; Marinus, J.

    2010-01-01

    Complex Regional Pain Syndrome (CRPS) usually develops after a noxious event, but spontaneous onsets have been described in 3-11% of the cases. The existence of spontaneous-onset CRPS is highly debated and the aim of the present study was therefore to compare the phenotypic characteristics of CRPS

  17. Complex Regional Pain Syndrome: An inflammatory disease

    NARCIS (Netherlands)

    M. Dirckx (Maaike)

    2015-01-01

    markdownabstractThe pathophysiology of Complex Regional Pain Syndrome (CRPS) is complex and still not completely understood. In addition to a convincing role of inflammation, there are a number of arguments why an involvement of the immune system has been suggested in the pathophysiology of CRPS.

  18. Medial tibial stress syndrome: a critical review

    NARCIS (Netherlands)

    Moen, Maarten H.; Tol, Johannes L.; Weir, Adam; Steunebrink, Miriam; de Winter, Theodorus C.

    2009-01-01

    Medial tibial stress syndrome (MTSS) is one of the most common leg injuries in athletes and soldiers. The incidence of MTSS is reported as being between 4% and 35% in military personnel and athletes. The name given to this condition refers to pain on the posteromedial tibial border during exercise,

  19. COMPLEX REGIONAL PAIN SYNDROME: AN UPDATE

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    Patta

    2015-10-01

    Full Text Available Complex Regional Pain Syndrome or CRPS is a chronic disabling heterogenous pain disorder due to trauma, resulting in sensory changes, motor function impairment, psychological changes. Diagnosis is based on clinical features and investigations. The patho - physiology is not clearly known. There is no single drug therapy, only combinations work. Treatment is multidisplinary involving medical, psychological and rehabi li tation. Newer modes of spinal cord stimulations, neuraxial mode of analgesics and newe r drugs are promising

  20. Brain in complex regional pain syndrome

    OpenAIRE

    Hotta, Jaakko

    2017-01-01

    Complex regional pain syndrome (CRPS) causes disabling and severe limb pain that is difficult to treat. The pain typically increases during motor actions, but is present also at rest. The pathophysiology of CRPS is incompletely understood. Some of the symptoms suggest involvement of the central nervous system, and accordingly, patients have been shown to display alterations in, for instance, the primary sensorimotor cortex (SM1) and indications of neuroinflammation. More thorough pathophysiol...

  1. Regional cerebral blood flow in Angelman syndrome

    International Nuclear Information System (INIS)

    Guecueyener, K.; Goekcora, N.; Ilgin, N.; Buyan, N.; Sayli, A.

    1993-01-01

    A patient with typical features of Angelman syndrome - a genetically inherited disorder involving developmental delay, ataxia, episodes of paroxysmal laughter and brachiocephaly - was studied with single-photon emission tomography. Hyperfusion found in the left frontal and left temporoparietal regions can provide insights into the functional cerebral pathology, which may be due to a disturbance of the developmental process related to a chromosomal abnormality. (orig.)

  2. Regional cerebral blood flow in Angelman syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Guecueyener, K [Dept. of Pediatric Neurology, Faculty of Medicine, Gazi Univ., Ankara (Turkey); Goekcora, N [Dept. of Nuclear Medicine, Faculty of Medicine, Gazi Univ., Ankara (Turkey); Ilgin, N [Dept. of Nuclear Medicine, Faculty of Medicine, Gazi Univ., Ankara (Turkey); Buyan, N [Dept. of Pediatric Neurology, Faculty of Medicine, Gazi Univ., Ankara (Turkey); Sayli, A [Dept. of Molecular Biology and Genetics, Faculty of Medicine, Gazi Univ., Ankara (Turkey)

    1993-07-01

    A patient with typical features of Angelman syndrome - a genetically inherited disorder involving developmental delay, ataxia, episodes of paroxysmal laughter and brachiocephaly - was studied with single-photon emission tomography. Hyperfusion found in the left frontal and left temporoparietal regions can provide insights into the functional cerebral pathology, which may be due to a disturbance of the developmental process related to a chromosomal abnormality. (orig.)

  3. Korsakoff’s syndrome: a critical review

    Directory of Open Access Journals (Sweden)

    Arts NJM

    2017-11-01

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

  4. Korsakoff’s syndrome: a critical review

    Science.gov (United States)

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

    2017-01-01

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

  5. New Concepts in Complex Regional Pain Syndrome

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    Tajerian, Maral; Clark, J David

    2015-01-01

    SYNOPSIS Despite the severe pain and disability associated with Complex Regional Pain Syndrome (CRPS), our lack of understanding of the pathophysiological mechanisms supporting this enigmatic condition prevents the rational design of new therapies, a situation that is frustrating both to the physician and the patient. The following review will highlight some of the mechanisms thought to be involved in the pathophysiology of CRPS in preclinical models and CRPS patients, with the ultimate goal that understanding these mechanisms will lead to the design of efficacious, mechanism-based treatments available to the clinic. PMID:26611388

  6. Medial tibial stress syndrome: a critical review.

    Science.gov (United States)

    Moen, Maarten H; Tol, Johannes L; Weir, Adam; Steunebrink, Miriam; De Winter, Theodorus C

    2009-01-01

    Medial tibial stress syndrome (MTSS) is one of the most common leg injuries in athletes and soldiers. The incidence of MTSS is reported as being between 4% and 35% in military personnel and athletes. The name given to this condition refers to pain on the posteromedial tibial border during exercise, with pain on palpation of the tibia over a length of at least 5 cm. Histological studies fail to provide evidence that MTSS is caused by periostitis as a result of traction. It is caused by bony resorption that outpaces bone formation of the tibial cortex. Evidence for this overloaded adaptation of the cortex is found in several studies describing MTSS findings on bone scan, magnetic resonance imaging (MRI), high-resolution computed tomography (CT) scan and dual energy x-ray absorptiometry. The diagnosis is made based on physical examination, although only one study has been conducted on this subject. Additional imaging such as bone, CT and MRI scans has been well studied but is of limited value. The prevalence of abnormal findings in asymptomatic subjects means that results should be interpreted with caution. Excessive pronation of the foot while standing and female sex were found to be intrinsic risk factors in multiple prospective studies. Other intrinsic risk factors found in single prospective studies are higher body mass index, greater internal and external ranges of hip motion, and calf girth. Previous history of MTSS was shown to be an extrinsic risk factor. The treatment of MTSS has been examined in three randomized controlled studies. In these studies rest is equal to any intervention. The use of neoprene or semi-rigid orthotics may help prevent MTSS, as evidenced by two large prospective studies.

  7. Hybridized Kibble-Zurek scaling in the driven critical dynamics across an overlapping critical region

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    Zhai, Liang-Jun; Wang, Huai-Yu; Yin, Shuai

    2018-04-01

    The conventional Kibble-Zurek scaling describes the scaling behavior in the driven dynamics across a single critical region. In this paper, we study the driven dynamics across an overlapping critical region, in which a critical region (Region A) is overlaid by another critical region (Region B). We develop a hybridized Kibble-Zurek scaling (HKZS) to characterize the scaling behavior in the driven process. According to the HKZS, the driven dynamics in the overlapping region can be described by the critical theories for both Region A and Region B simultaneously. This results in a constraint on the scaling function in the overlapping critical region. We take the quantum Ising chain in an imaginary longitudinal field as an example. In this model, the critical region of the Yang-Lee edge singularity and the critical region of the ferromagnetic-paramagnetic phase transition overlap with each other. We numerically confirm the HKZS by simulating the driven dynamics in this overlapping critical region. The HKZSs in other models are also discussed.

  8. [Complex regional pain syndrome (CRPS) : An update].

    Science.gov (United States)

    Dimova, V; Birklein, F

    2018-04-17

    The acute phase of complex regional pain syndrome (CRPS) is pathophysiologically characterized by an activation of the immune system and its associated inflammatory response. During the course of CRPS, central nervous symptoms like mechanical hyperalgesia, loss of sensation, and body perception disorders develop. Psychological factors such as pain-related anxiety and traumatic events might have a negative effect on the treatment outcome. While the visible inflammatory symptoms improve, the pain often persists. A stage adapted, targeted treatment could improve the prognosis. Effective multidisciplinary treatment includes the following: pharmacotherapy with steroids, bisphosphonates, or dimethylsulfoxide cream (acute phase), and antineuropathic analgesics (all phases); physiotherapy and behavioral therapy for pain-related anxiety and avoidance of movement; and interventional treatment like spinal cord or dorsal root ganglion stimulation if noninvasive options failed.

  9. Is refeeding syndrome relevant for critically ill patients?

    Science.gov (United States)

    Koekkoek, Wilhelmina A C; Van Zanten, Arthur R H

    2018-03-01

    To summarize recent relevant studies regarding refeeding syndrome (RFS) in critically ill patients and provide recommendations for clinical practice. Recent knowledge regarding epidemiology of refeeding syndrome among critically ill patients, how to identify ICU patients at risk, and strategies to reduce the potential negative impact on outcome are discussed. RFS is a potentially fatal acute metabolic derangement that ultimately can result in marked morbidity and even mortality. These metabolic derangements in ICU patients differ from otherwise healthy patients with RFS, as there is lack of anabolism. This is because of external stressors inducing a hypercatabolic response among other reasons also reflected by persistent high glucagon despite initiation of feeding. Lack of a proper uniform definition complicates diagnosis and research of RFS. However, refeeding hypophosphatemia is commonly encountered during critical illness. The correlations between risk factors proposed by international guidelines and the occurrence of RFS in ICU patients remains unclear. Therefore, regular phosphate monitoring is recommended. Based on recent trials among critically ill patients, only treatment with supplementation of electrolytes and vitamins seems not sufficient. In addition, caloric restriction for several days and gradual increase of caloric intake over days is recommendable.

  10. Migrant Cuisine, Critical Regionalism and Gastropoetics

    Directory of Open Access Journals (Sweden)

    Ben Highmore

    2013-02-01

    Full Text Available This essay is based around two sentences from Nadeem Alslam’s 2004 novel Maps for Lost Lovers. From this base comes an exploration of what aesthetics could mean for cultural studies, and what sorts of practices it could foster. The essay argues for the pertinence this may have for the study of food culture, while also taking up the project of ‘critical regionalism’ and ‘gastropoetics’ as a way of moving from the tightly figured frame of a fragment from a novel, to the histories, geographies and affects that impact on it.

  11. Rethinking the Psychogenic Model of Complex Regional Pain Syndrome: Somatoform Disorders and Complex Regional Pain Syndrome

    Science.gov (United States)

    Hill, Renee J.; Chopra, Pradeep; Richardi, Toni

    2012-01-01

    Abstract Explaining the etiology of Complex Regional Pain Syndrome (CRPS) from the psychogenic model is exceedingly unsophisticated, because neurocognitive deficits, neuroanatomical abnormalities, and distortions in cognitive mapping are features of CRPS pathology. More importantly, many people who have developed CRPS have no history of mental illness. The psychogenic model offers comfort to physicians and mental health practitioners (MHPs) who have difficulty understanding pain maintained by newly uncovered neuro inflammatory processes. With increased education about CRPS through a biopsychosocial perspective, both physicians and MHPs can better diagnose, treat, and manage CRPS symptomatology. PMID:24223338

  12. Inflammation in complex regional pain syndrome

    Science.gov (United States)

    Parkitny, Luke; McAuley, James H.; Di Pietro, Flavia; Stanton, Tasha R.; O’Connell, Neil E.; Marinus, Johan; van Hilten, Jacobus J.

    2013-01-01

    Objectives: We conducted a systematic review of the literature with meta-analysis to determine whether complex regional pain syndrome (CRPS) is associated with a specific inflammatory profile and whether this is dependent on the duration of the condition. Methods: Comprehensive searches of the literature using MEDLINE, Embase, Scopus, Web of Science, and reference lists from published reviews identified articles that measured inflammatory factors in CRPS. Two independent investigators screened titles and abstracts, and performed data extraction and risk of bias assessments. Studies were subgrouped by medium (blood, blister fluid, and CSF) and duration (acute and chronic CRPS). Where possible, meta-analyses of inflammatory factor concentrations were performed and pooled effect sizes were calculated using random-effects models. Results: Twenty-two studies were included in the systematic review and 15 in the meta-analysis. In acute CRPS, the concentrations of interleukin (IL)-8 and soluble tumor necrosis factor receptors I (sTNF-RI) and II (sTNF-RII) were significantly increased in blood. In chronic CRPS, significant increases were found in 1) TNFα, bradykinin, sIL-1RI, IL-1Ra, IL-2, sIL-2Ra, IL-4, IL-7, interferon-γ, monocyte chemoattractant protein-1 (MCP-1), and sRAGE (soluble receptor for advanced glycation end products) in blood; 2) IL-1Ra, MCP-1, MIP-1β, and IL-6 in blister fluid; and 3) IL-1β and IL-6 in CSF. Chronic CRPS was also associated with significantly decreased 1) substance P, sE-selectin, sL-selectin, sP-selectin, and sGP130 in blood; and 2) soluble intercellular adhesion molecule-1 (sICAM-1) in CSF. Most studies failed to meet 3 or more of our quality criteria. Conclusion: CRPS is associated with the presence of a proinflammatory state in the blood, blister fluid, and CSF. Different inflammatory profiles were found for acute and chronic cases. PMID:23267031

  13. A critical review of regional economic integration in China

    OpenAIRE

    Wang RUI

    2015-01-01

    Under the circumstances of economic globalization, regional economic integration has become the mainstream of current economic development for each country, so China has to pay more attention to it. The critical review on regional economic integration in China can lay a certain foundation and provide experience for the in-depth research. Main contents of regional economic integration are refined according to the previous studies and realities, including the integration of regional economic re...

  14. Saturated properties prediction in critical region by a quartic ...

    African Journals Online (AJOL)

    A diverse substance library containing extensive PVT data for 77 pure components was used to critically evaluate the performance of a quartic equation of state and other four famous cubic equations of state in critical region. The quartic EOS studied in this work was found to significantly superior to the others in both vapor ...

  15. COMPLEX REGIONAL PAIN SYNDROME – CLINIC, DIAGNOSTICS, TREATMENT

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2014-07-01

    Full Text Available The problem of pain today remains one of the fundamental issues of medical care. It is known that the pain is the leading cause of treatmentto the doctor. Among pain syndromes, a special place belongs to the complex regional pain syndromes. They are distinguished by the multidisciplinary problem, the presence of explicit nature of the pain, difficulty of diagnosis and nature of the treatment. The article presentsmodern data on the diagnosis and treatment of the complex regional pain syndromes.

  16. COMPLEX REGIONAL PAIN SYNDROME – CLINIC, DIAGNOSTICS, TREATMENT

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2013-01-01

    Full Text Available The problem of pain today remains one of the fundamental issues of medical care. It is known that the pain is the leading cause of treatmentto the doctor. Among pain syndromes, a special place belongs to the complex regional pain syndromes. They are distinguished by the multidisciplinary problem, the presence of explicit nature of the pain, difficulty of diagnosis and nature of the treatment. The article presentsmodern data on the diagnosis and treatment of the complex regional pain syndromes.

  17. Submicroscopic duplication of the Wolf-Hirschhorn critical region with a 4p terminal deletion.

    Science.gov (United States)

    Roselló, M; Monfort, S; Orellana, C; Ferrer-Bolufer, I; Quiroga, R; Oltra, S; Martínez, F

    2009-01-01

    Chromosomal rearrangements in the short arm of chromosome 4 can result in 2 different clinical entities: Wolf-Hirschhorn syndrome (WHS), characterized by severe growth delay, mental retardation, microcephaly, 'Greek helmet' facies, and closure defects, or partial 4p trisomy, associated with multiple congenital anomalies, mental retardation, and facial dysmorphisms. We present clinical and laboratory findings in a patient who showed a small duplication in 4p16.3 associated with a subtle terminal deletion in the same chromosomal region. GTG-banding analyses, multiplex ligation-dependent probe amplification analyses, and studies by array-based comparative genomic hybridization were performed. The results of the analyses revealed a de novo 1.3 Mb deletion of the terminal 4p and a 1.1 Mb duplication in our patient, encompassing the WHS critical region. Interestingly, this unusual duplication/deletion rearrangement results in an intermediate phenotype that shares characteristics of the WHS and the 4p trisomy syndrome. The use of novel technologies in the genetic diagnosis leads to the description of new clinical syndromes; there is a growing list of microduplication syndromes. Therefore, we propose that overexpression of candidate genes in WHS (WHSC1, WHSC2 and LETM1) due to a duplication causes a clinical entity different to both the WHS and 4p trisomy syndrome. (c) 2009 S. Karger AG, Basel.

  18. Teaching Critical Thinking in World Regional Geography through Stakeholder Debate

    Science.gov (United States)

    Sziarto, Kristin M.; McCarthy, Linda; Padilla, Nicholas L.

    2014-01-01

    Using a stakeholder debate based on a real-world case of regional construction--that of Turkey's application to join the European Union--improved students' critical thinking in an introductory world regional geography course. Such courses are a staple offering among US geography departments, and often the only exposure of non-majors to geographic…

  19. Organization and annotation of the Xcat critical region: elimination of seven positional candidate genes.

    Science.gov (United States)

    Huang, Kristen M; Geunes-Boyer, Scarlett; Wu, Sufen; Dutra, Amalia; Favor, Jack; Stambolian, Dwight

    2004-05-01

    Xcat mice display X-linked congenital cataracts and are a mouse model for the human X-linked cataract disease Nance Horan syndrome (NHS). The genetic defect in Xcat mice and NHS patients is not known. We isolated and sequenced a BAC contig representing a portion of the Xcat critical region. We combined our sequencing data with the most recent mouse sequence assemblies from both Celera and public databases. The sequence of the 2.2-Mb Xcat critical region was then analyzed for potential Xcat candidate genes. The coding regions of the seven known genes within this area (Rai2, Rbbp7, Ctps2, Calb3, Grpr, Reps2, and Syap1) were sequenced in Xcat mice and no mutations were detected. The expression of Rai2 was quantitatively identical in wild-type and Xcat mutant eyes. These results indicate that the Xcat mutation is within a novel, undiscovered gene.

  20. Critical scattering of neutrons by Fe: study of the hydrodynamic and critical regions

    International Nuclear Information System (INIS)

    Parette, Georges

    1971-01-01

    In the present work we describe the latest experiments on the critical magnetic scattering of neutrons by iron just above the Curie temperature, performed at the Centre d'Etudes Nucleaires at Saclay. In these experiments we have tried to explore the 'hydrodynamical region' as defined by the 'scaling laws' and to determine the temperature dependence of the diffusion constant. These experiments yield a verification of the recent theoretical calculations made by P. Resibois and C. Piette. These calculations and several measurements which we have conducted show the existence of an intermediate region between the 'critical' and the 'hydrodynamical' regions, which we call the 'quasi-hydrodynamical' region. In the hydrodynamical region, whose borders are well defined by the calculations of Resibois and Piette, our results confirm the theoretical predictions concerning this region. (author) [fr

  1. Two unique patients with novel microdeletions in 4p16.3 that exclude the WHS critical regions: implications for critical region designation.

    Science.gov (United States)

    South, Sarah T; Bleyl, Steven B; Carey, John C

    2007-09-15

    Wolf-Hirschhorn syndrome (WHS) is characterized by growth delay, developmental delay, hypotonia, seizures, feeding difficulties, and characteristic facial features. Deletion of either of two critical regions (WHSCR and WHSCR-2) within chromosome band 4p16.3 has been proposed as necessary for the minimal clinical manifestations of WHS and controversy remains regarding their designation. We describe two patients with novel terminal microdeletions in 4p16.3 who lack the characteristic facial features but do show some of the more nonspecific manifestations of WHS. The first patient had a ring chromosome 4 with an intact 4q subtelomere and a terminal 4p microdeletion of approximately 1.27-1.46 Mb. This deletion was distal to both proposed critical regions. The second patient had a normal karyotype with a terminal 4p microdeletion of approximately 1.78 Mb. This deletion was distal to WHSCR and the breakpoint was near or within the known distal boundary for WHSCR-2. Both patients showed significant postnatal growth delay, mild developmental delays and feeding difficulties. Their facial features were not typical for WHS. The phenotype of the first patient may have been influenced by the presence of a ring chromosome. Seizures were absent in the first patient whereas the second patient had a complex seizure disorder. Characterization of these patients supports the hypothesis that a gene in WHSCR-2, LETM1, plays a direct role in seizure development, and demonstrates that components of the WHS phenotype can be seen with deletions distal to the known boundaries of the two proposed critical regions. These patients also emphasize the difficulty of mapping clinical manifestations common to many aneusomy syndromes. (c) 2007 Wiley-Liss, Inc.

  2. 4p16.3 microdeletions and microduplications detected by chromosomal microarray analysis: New insights into mechanisms and critical regions.

    Science.gov (United States)

    Bi, Weimin; Cheung, Sau-Wai; Breman, Amy M; Bacino, Carlos A

    2016-10-01

    Deletions in the 4p16.3 region cause Wolf-Hirschhorn syndrome, a well known contiguous microdeletion syndrome with the critical region for common phenotype mapped in WHSCR2. Recently, duplications in 4p16.3 were reported in three patients with developmental delay and dysmorphic features. Through chromosomal microarray analysis, we identified 156 patients with a deletion (n = 109) or duplication (n = 47) in 4p16.3 out of approximately 60,000 patients analyzed by Baylor Miraca Genetics Laboratories. Seventy-five of the postnatally detected deletions encompassed the entire critical region, 32 (43%) of which were associated with other chromosome rearrangements, including six patients (8%) that had a duplication adjacent to the terminal deletion. Our data indicate that Wolf-Hirschhorn syndrome deletions with an adjacent duplication occur at a higher frequency than previously appreciated. Pure deletions (n = 14) or duplications (n = 15) without other copy number changes distal to or inside the WHSCR2 were identified for mapping of critical regions. Our data suggest that deletion of the segment from 0.6 to 0.9 Mb from the terminus of 4p causes a seizure phenotype and duplications of a region distal to the previously defined smallest region of overlap for 4p16.3 microduplication syndrome are associated with neurodevelopmental problems. We detected seven Wolf-Hirschhorn syndrome deletions and one 4p16.3 duplication prenatally; all of the seven are either >8 Mb in size and/or associated with large duplications. In conclusion, our study provides deeper insight into the molecular mechanisms, the critical regions and effective prenatal diagnosis for 4p16.3 deletions/ duplications. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  3. Impact of caloric intake in critically ill patients with, and without, refeeding syndrome: A retrospective study.

    Science.gov (United States)

    Olthof, Laura E; Koekkoek, W A C Kristine; van Setten, Coralien; Kars, Johannes C N; van Blokland, Dick; van Zanten, Arthur R H

    2017-08-10

    Refeeding syndrome comprises metabolic disturbances that occur after the reintroduction of feeding after prolonged fasting. Standard care consists of correcting fluid and electrolytes imbalances. Energy intake during refeeding syndrome is heavily debated. This study addresses the effect of caloric intake on outcome during the management of refeeding syndrome. A retrospective study among critically ill invasive mechanically ventilated patients admitted for >7 days to a medical-surgical ICU. Refeeding syndrome was diagnosed by the occurrence of new onset hypophosphatemia (refeeding syndrome were compared and subgroup analysis on energy intake within the refeeding population was performed for the duration of survival. Of 337 enrolled patients, 124 (36.8%) developed refeeding syndrome and 213 patients (63.2%) maintained normal serum phosphate levels. Between the two groups, no statistical significant differences in clinical outcomes were observed. Within the refeeding syndrome group, a reduced 6-month mortality risk for low caloric intake (Refeeding syndrome is common among prolonged mechanically ventilated critically ill patients, however not predictable by baseline characteristics. Among patients that develop refeeding syndrome low caloric intake was associated with a reduction in 6-month mortality risk. This effect was not seen in patients without refeeding syndrome. Findings support caloric restriction in refeeding syndrome during critical illness. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  4. LETM1, a novel gene encoding a putative EF-hand Ca(2+)-binding protein, flanks the Wolf-Hirschhorn syndrome (WHS) critical region and is deleted in most WHS patients.

    Science.gov (United States)

    Endele, S; Fuhry, M; Pak, S J; Zabel, B U; Winterpacht, A

    1999-09-01

    Deletions within human chromosome 4p16.3 cause Wolf-Hirschhorn syndrome (WHS), which is characterized by severe mental and developmental defects. It is thought that haploinsufficiency of more than one gene contributes to the complex phenotype. We have cloned and characterized a novel gene (LETM1) that is deleted in nearly all WHS patients. LETM1 encodes a putative member of the EF-hand family of Ca(2+)-binding proteins. The protein contains two EF-hands, a transmembrane domain, a leucine zipper, and several coiled-coil domains. On the basis of its possible Ca(2+)-binding property and involvement in Ca(2+) signaling and/or homeostasis, we propose that haploinsufficiency of LETM1 may contribute to the neuromuscular features of WHS patients. Copyright 1999 Academic Press.

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

    Science.gov (United States)

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

    2012-06-01

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

  6. Complex Regional Pain Syndrome in Children: Asking the Right Questions

    Directory of Open Access Journals (Sweden)

    Kenneth R Goldschneider

    2012-01-01

    Full Text Available BACKGROUND: Complex regional pain syndrome (CRPS is a painful disorder without a known unifying mechanism. There are little data on which to base evaluation and treatment decisions, and what data are available come from studies involving adults; however, even that literature is relatively sparse. Developing robust research for CRPS in children is essential for the progress toward optimal treatment.

  7. Genetic and epidemiological aspect of Complex Regional Pain Syndrome

    NARCIS (Netherlands)

    Rooij, Annetje Monique de

    2010-01-01

    Complex Regional Pain Syndrome (CRPS) is a painful disorder affecting one or more extremities. CRPS is characterized by various combinations of sensory, autonomic and motor disturbances. Genetic factors are suggested to play a role in CRPS, but this has not been extensively studied. Therefore the

  8. Neuroimmune Alterations in the Complex Regional Pain Syndrome

    NARCIS (Netherlands)

    F.J.P.M. Huygen (Frank)

    2004-01-01

    textabstractComplex Regional Pain Syndrome (CRPS) is a disease which occurs as a complication after surgery or trauma, although spontaneous development is described. CRPS is characterized by continuing pain, sensory and vasomotor, sudomotor, motor and trophic disturbances. Many of these symptoms are

  9. Reflex sympathetic dystrophy/complex regional pain syndrome, type 1

    African Journals Online (AJOL)

    Enrique

    with MRI every 3 months and the bone marrow oedema disappeared after 6 months. Introduction ... SA JOURNAL OF RADIOLOGY • August 2004. Reflex sympathetic dystrophy/complex regional pain syndrome, type 1 ... may be either trauma of external origin or iatrogenic, post surgery. In some patients particularly children ...

  10. [Complex regional pain syndrome type 1: negating the myth

    NARCIS (Netherlands)

    Frolke, J.P.M.; Dongen, R.T.M. van; Meent, H. van de

    2015-01-01

    Complex regional pain syndrome type 1 (CRPS-1) was identified in the Netherlands more than 30 years ago, but since then the arguments supporting this diagnosis have become weaker. Incidence has decreased, it is often not possible to make a definite diagnosis, the pathophysiology remains unclear and

  11. Evidence based guidelines for complex regional pain syndrome type 1

    NARCIS (Netherlands)

    Perez, R.S.G.M.; Zollinger, P.E.; Dijkstra, P.U.; Thomassen-Hilgersom, I.L.; Zuurmond, W.W.A.; Rosenbrand, C.J.G.M.; Geerzen, J.H.B.

    2010-01-01

    Background: Treatment of complex regional pain syndrome type I (CRPS-I) is subject to discussion. The purpose of this study was to develop multidisciplinary guidelines for treatment of CRPS-I.Method: A multidisciplinary task force graded literature evaluating treatment effects for CRPS-I according

  12. Evidence based guidelines for complex regional pain syndrome type 1

    NARCIS (Netherlands)

    Perez, Roberto S.; Zollinger, Paul E.; Dijkstra, Pieter U.; Thomassen-Hilgersom, Ilona L.; Zuurmond, Wouter W.; Rosenbrand, Kitty C. J.; Geertzen, Jan H.

    2010-01-01

    Background: Treatment of complex regional pain syndrome type I (CRPS-I) is subject to discussion. The purpose of this study was to develop multidisciplinary guidelines for treatment of CRPS-I. Method: A multidisciplinary task force graded literature evaluating treatment effects for CRPS-I according

  13. Criticality Analysis of SFP Region I under Dry Air Condition

    International Nuclear Information System (INIS)

    Kim, Ki Yong; Kim, Min Chul

    2016-01-01

    This paper is to provide a result of the criticality evaluation under the condition that new fuel assemblies for initial fuel loading are storing in Region 1 of SFP in the dry air. The objective of this analysis is to ensure the effective neutron multiplication factor(k_e_f_f) of SFP is less than 0.95 under that condition. This analysis ensured the effective neutron multiplication factor(k_e_f_f) of Region 1 of SFP is less than 0.95 under the condition in the air. The keff in Region I of SFP under the condition of the dry air is 0.5865. The increased k_c_a_l_c of the Region 1 after the mislocated fuel assembly accident is 0.0444 at the pool flooded with un-borated water

  14. Criticality analyses of regions containing uranium in the earth history

    International Nuclear Information System (INIS)

    Ravnik, M.

    2005-01-01

    Investigations of necessary conditions for a self-sustained chain reaction in the Earth inner regions hypothetically containing uranium is presented for the time interval from Earth formation to present time. It is determined that criticality was theoretically possible up to 2.5 Ga before present if uranium concentrated in pure form. In the early geological history (4 Ga before present) the self-sustained criticality could occur even if uranium was diluted up to 1:20 by the average core material or 1:60 by the average mantle material. If other metallic materials of similar density as uranium (e.g., Au, W) or similar atomic weight (e.g., Th) concentrated from the primordial mixture in equal proportion as uranium, criticality was not possible for any period in Earth history provided that the basic material contained no light nuclides (H, C). Criticality in the Earth inner regions could have established only if uranium concentrated from the basic material more effectively than elements of similar density or atomic number. (orig.)

  15. Simple Model for Identifying Critical Regions in Atrial Fibrillation

    Science.gov (United States)

    Christensen, Kim; Manani, Kishan A.; Peters, Nicholas S.

    2015-01-01

    Atrial fibrillation (AF) is the most common abnormal heart rhythm and the single biggest cause of stroke. Ablation, destroying regions of the atria, is applied largely empirically and can be curative but with a disappointing clinical success rate. We design a simple model of activation wave front propagation on an anisotropic structure mimicking the branching network of heart muscle cells. This integration of phenomenological dynamics and pertinent structure shows how AF emerges spontaneously when the transverse cell-to-cell coupling decreases, as occurs with age, beyond a threshold value. We identify critical regions responsible for the initiation and maintenance of AF, the ablation of which terminates AF. The simplicity of the model allows us to calculate analytically the risk of arrhythmia and express the threshold value of transversal cell-to-cell coupling as a function of the model parameters. This threshold value decreases with increasing refractory period by reducing the number of critical regions which can initiate and sustain microreentrant circuits. These biologically testable predictions might inform ablation therapies and arrhythmic risk assessment.

  16. Regional cerebral glucose metabolism in patients with alcoholic Korsakoff's syndrome

    International Nuclear Information System (INIS)

    Kessler, R.M.; Parker, E.S.; Clark, C.M.; Martin, P.R.; George, D.T.; Weingartner, H.; Sokoloff, L.; Ebert, M.H.; Mishkin, M.

    1985-01-01

    Seven alcoholic male subjects diagnosed as having Korsakoff's syndrome and eight age-matched male normal volunteers were studied with /sup 18/F 2-fluoro-2-deoxy-D-glucose (2/sup 18/FDG). All subjects were examined at rest with eyes covered in a quiet, darkened room. Serial plasma samples were obtained following injection of 4 to 5 mCi of 2/sup 18/FDG. Tomographic slices spaced at 10mm axial increments were obtained (in-plane resolution = 1.75 cm, axial resolution = 1.78 cm). Four planes were selected from each subject, and a total of 46 regions of interest were sampled and glucose metabolic rates for each region calculated. The mean glucose metalbolic rate for the 46 regions in the Korsakoff subjects was significantly lower than that in the normal controls (5.17 +- .43 versus 6.6 +- 1.31). A Q-component analysis, which examined each subject's regional rates relative to his mean rate, revealed two distinct patterns in the Korsakoff group. Glucose metabolism was significantly reduced in 37 of the 46 regions sampled. Reduced cerebral glucose metabolism in a nondemented group of subjects has not previously been reported. The reduction in cortical metabolism may be the result of damage to sub-cortical projecting systems. The differing patterns of cerebral metabolism in Korsakoff's syndrome suggests subgroups with differing neuropathology. Regions implicated in memory function, medial temporal, thalamic and medial prefrontal were among the regions reduced in metabolism

  17. Metabolic syndrome in the Mediterranean region: Current status

    OpenAIRE

    Panagiotis Anagnostis

    2012-01-01

    Metabolic syndrome (MetS) is a cluster of metabolic abnormalities including abdominal obesity, impaired fasting glucose, hypertension and dyslipidemia. It seems to affect about one-fourth to one-fifth of the Mediterranean population, and its prevalence increases with age, being similar for both sexes and depending on the region and the definition used, with the National Cholesterol Education Program-Adult Treatment Panel-III (NCEP-ATPIII) definition being the most effective in the identificat...

  18. Burnout syndrome in oral and maxillofacial surgeons: a critical analysis.

    Science.gov (United States)

    Porto, G G; Carneiro, S C; Vasconcelos, B C; Nascimento, M M; Leal, J L F

    2014-07-01

    The aim of this study was to determine the prevalence of burnout syndrome among Brazilian oral and maxillofacial surgeons and its relationship with socio-demographic, clinical, and habit variables. The sample of this study comprised 116 surgeons. The syndrome was quantified using the Maslach Burnout Inventory (General Survey), which defines burnout as the triad of high emotional exhaustion, high depersonalization, and low personal accomplishment. The criteria of Grunfeld et al. were used to evaluate the presence of the syndrome (17.2%). No significant differences between the surgeons diagnosed with and without the syndrome were observed according to age (P=0.804), sex (P=0.197), marital status (P=0.238), number of children (P=0.336), years of professional experience (P=0.102), patients attended per day (P=0.735), hours worked per week (P=0.350), use of alcohol (P=0.148), sports practice (P=0.243), hobbies (P=0.161), or vacation period per year (P=0.215). Significant differences occurred in the variables sex in the emotional exhaustion subscale (P=0.002) and use or not of alcohol in the personal accomplishment subscale (P=0.035). Burnout syndrome among Brazilian surgeons is average, showing a low personal accomplishment. Copyright © 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  19. Complex regional pain syndrome type I following pacemaker implantation

    Directory of Open Access Journals (Sweden)

    Sangita Kamath

    2015-12-01

    Full Text Available A 70-year-old woman presented with burning pain and swelling over dorsum of right hand and small joints of the fingers, associated with redness, feeling of warmth, and stiffness of the fingers, with inability to bend the fingers since 2 months. The symptoms were progressively increasing in intensity for the past 1 month. There was no history of fever or trauma to the hand. Two months before her symptoms started, she had permanent pacemaker implanted for complete heart block with syncope. She was hypertensive and was on regular medication. Her X-ray of right hand showed decreased bone density (demineralisation, suggestive of osteopenia. A diagnosis of reflex sympathetic dystrophy syndrome or complex regional pain syndrome type I induced by pacemaker insertion was made. She was treated with amitriptyline and steroids, after which her symptoms improved dramatically.

  20. A study of fluid alkali metals in the critical region

    International Nuclear Information System (INIS)

    Balasubramanian, R.

    2006-01-01

    On the basis of the generalised van der Waals equation of state, Riedel's thermodynamic similarity parameter, a measure of the temperature dependence of vapour pressure in the critical region is determined for caesium, rubidium and potassium. This generalised equation differs from the known van der Waals equation of state by the modified expression for molecular pressure. The results of determination of Riedel's thermodynamic similarity parameter of caesium, rubidium and potassium are in good agreement with experimental data. Moreover, the given generalised van der Waals equation of state yields a better fit with experimental data on Riedel's thermodynamic similarity parameter for fluid alkali metals when compared with other correlations such as Van Ness and Abbott equation, Pitzer expansion, Pitzer acentric factor correlation, modified Rackett technique, Lee-Kesler vapour pressure relation and Clausius-Clayperon equation

  1. A study of fluid alkali metals in the critical region

    Energy Technology Data Exchange (ETDEWEB)

    Balasubramanian, R. [Department of Physics, Kongu Engineering College, Perundurai, Erode 638 052, Tamil Nadu (India)]. E-mail: drrbala@yahoo.com

    2006-05-31

    On the basis of the generalised van der Waals equation of state, Riedel's thermodynamic similarity parameter, a measure of the temperature dependence of vapour pressure in the critical region is determined for caesium, rubidium and potassium. This generalised equation differs from the known van der Waals equation of state by the modified expression for molecular pressure. The results of determination of Riedel's thermodynamic similarity parameter of caesium, rubidium and potassium are in good agreement with experimental data. Moreover, the given generalised van der Waals equation of state yields a better fit with experimental data on Riedel's thermodynamic similarity parameter for fluid alkali metals when compared with other correlations such as Van Ness and Abbott equation, Pitzer expansion, Pitzer acentric factor correlation, modified Rackett technique, Lee-Kesler vapour pressure relation and Clausius-Clayperon equation.

  2. Critical review of studies on atmospheric dispersion in coastal regions

    International Nuclear Information System (INIS)

    Shearer, D.L.; Kaleel, R.J.

    1982-09-01

    This study effort was required as a preliminary step prior to initiation of field measurements of atmospheric dispersion in coastal regions. The Nuclear Regulatory Commission (NRC) is in the process of planning an extensive field measurement program to generate data which will serve as improved data bases for licensing decisions, confirmation of regulations, standards, and guides, and for site characterizations. The study being reported here is an effort directed to obtaining as much information as is possible from existing studies that is relevant toward NRC's objectives. For this study, reports covering research and meteorological measurements conducted for industrial purposes, utility needs, military objectives, and academic studies were obtained and critically reviewed in light of NRC's current data needs. This report provides an interpretation of the extent of existing usable information, an indication of the potential for tailoring existing research toward current NRC information needs, and recommendations for several follow-on studies which could provide valuable additional information through reanalysis of the data. Recommendations are also offered regarding new measurement programs. Emphasis is placed on the identification and acquisition of data from atmospheric tracer studies conducted in coastal regions. A total of 225 references were identified which deal with the coastal atmosphere, including meteorological and tracer measurement programs, theoretical descriptions of the relevant processes, and dispersion models

  3. Defining recovery in chronic fatigue syndrome: a critical review.

    Science.gov (United States)

    Adamowicz, Jenna L; Caikauskaite, Indre; Friedberg, Fred

    2014-11-01

    In chronic fatigue syndrome (CFS), the lack of consensus on how recovery should be defined or interpreted has generated controversy and confusion. The purpose of this paper was to systematically review, compare, and evaluate the definitions of recovery reported in the CFS literature and to make recommendations about the scope of recovery assessments. A search was done using the MEDLINE, PubMed, PsycINFO, CINAHL, and Cochrane databases for peer review papers that contained the search terms "chronic fatigue syndrome" and "recovery," "reversal," "remission," and/or "treatment response." From the 22 extracted studies, recovery was operationally defined by reference with one or more of these domains: (1) pre-morbid functioning; (2) both fatigue and function; (3) fatigue (or related symptoms) alone; (4) function alone; and/or (5) brief global assessment. Almost all of the studies measuring recovery in CFS did so differently. The brief global assessment was the most common outcome measure used to define recovery. Estimates of recovery ranged from 0 to 66 % in intervention studies and 2.6 to 62 % in naturalistic studies. Given that the term "recovery" was often based on limited assessments and less than full restoration of health, other more precise and accurate labels (e.g., clinically significant improvement) may be more appropriate and informative. In keeping with common understandings of the term recovery, we recommend a consistent definition that captures a broad-based return to health with assessments of both fatigue and function as well as the patient's perceptions of his/her recovery status.

  4. A Critical Care Societies Collaborative Statement: Burnout Syndrome in Critical Care Health-care Professionals. A Call for Action.

    Science.gov (United States)

    Moss, Marc; Good, Vicki S; Gozal, David; Kleinpell, Ruth; Sessler, Curtis N

    2016-07-01

    Burnout syndrome (BOS) occurs in all types of health-care professionals and is especially common in individuals who care for critically ill patients. The development of BOS is related to an imbalance of personal characteristics of the employee and work-related issues or other organizational factors. BOS is associated with many deleterious consequences, including increased rates of job turnover, reduced patient satisfaction, and decreased quality of care. BOS also directly affects the mental health and physical well-being of the many critical care physicians, nurses, and other health-care professionals who practice worldwide. Until recently, BOS and other psychological disorders in critical care health-care professionals remained relatively unrecognized. To raise awareness of BOS, the Critical Care Societies Collaborative (CCSC) developed this call to action. The present article reviews the diagnostic criteria, prevalence, causative factors, and consequences of BOS. It also discusses potential interventions that may be used to prevent and treat BOS. Finally, we urge multiple stakeholders to help mitigate the development of BOS in critical care health-care professionals and diminish the harmful consequences of BOS, both for critical care health-care professionals and for patients.

  5. Genome-Wide Expression Profiling of Complex Regional Pain Syndrome

    Science.gov (United States)

    Jin, Eun-Heui; Zhang, Enji; Ko, Youngkwon; Sim, Woo Seog; Moon, Dong Eon; Yoon, Keon Jung; Hong, Jang Hee; Lee, Won Hyung

    2013-01-01

    Complex regional pain syndrome (CRPS) is a chronic, progressive, and devastating pain syndrome characterized by spontaneous pain, hyperalgesia, allodynia, altered skin temperature, and motor dysfunction. Although previous gene expression profiling studies have been conducted in animal pain models, there genome-wide expression profiling in the whole blood of CRPS patients has not been reported yet. Here, we successfully identified certain pain-related genes through genome-wide expression profiling in the blood from CRPS patients. We found that 80 genes were differentially expressed between 4 CRPS patients (2 CRPS I and 2 CRPS II) and 5 controls (cut-off value: 1.5-fold change and pCRPS patients and 18 controls by quantitative reverse transcription-polymerase chain reaction (qRT-PCR). We focused on the MMP9 gene that, by qRT-PCR, showed a statistically significant difference in expression in CRPS patients compared to controls with the highest relative fold change (4.0±1.23 times and p = 1.4×10−4). The up-regulation of MMP9 gene in the blood may be related to the pain progression in CRPS patients. Our findings, which offer a valuable contribution to the understanding of the differential gene expression in CRPS may help in the understanding of the pathophysiology of CRPS pain progression. PMID:24244504

  6. Critically Ill Patients With the Middle East Respiratory Syndrome: A Multicenter Retrospective Cohort Study.

    Science.gov (United States)

    Arabi, Yaseen M; Al-Omari, Awad; Mandourah, Yasser; Al-Hameed, Fahad; Sindi, Anees A; Alraddadi, Basem; Shalhoub, Sarah; Almotairi, Abdullah; Al Khatib, Kasim; Abdulmomen, Ahmed; Qushmaq, Ismael; Mady, Ahmed; Solaiman, Othman; Al-Aithan, Abdulsalam M; Al-Raddadi, Rajaa; Ragab, Ahmed; Al Mekhlafi, Ghaleb A; Al Harthy, Abdulrahman; Kharaba, Ayman; Ahmadi, Mashael Al; Sadat, Musharaf; Mutairi, Hanan Al; Qasim, Eman Al; Jose, Jesna; Nasim, Maliha; Al-Dawood, Abdulaziz; Merson, Laura; Fowler, Robert; Hayden, Frederick G; Balkhy, Hanan H

    2017-10-01

    To describe patient characteristics, clinical manifestations, disease course including viral replication patterns, and outcomes of critically ill patients with severe acute respiratory infection from the Middle East respiratory syndrome and to compare these features with patients with severe acute respiratory infection due to other etiologies. Retrospective cohort study. Patients admitted to ICUs in 14 Saudi Arabian hospitals. Critically ill patients with laboratory-confirmed Middle East respiratory syndrome severe acute respiratory infection (n = 330) admitted between September 2012 and October 2015 were compared to consecutive critically ill patients with community-acquired severe acute respiratory infection of non-Middle East respiratory syndrome etiology (non-Middle East respiratory syndrome severe acute respiratory infection) (n = 222). None. Although Middle East respiratory syndrome severe acute respiratory infection patients were younger than those with non-Middle East respiratory syndrome severe acute respiratory infection (median [quartile 1, quartile 3] 58 yr [44, 69] vs 70 [52, 78]; p < 0.001), clinical presentations and comorbidities overlapped substantially. Patients with Middle East respiratory syndrome severe acute respiratory infection had more severe hypoxemic respiratory failure (PaO2/FIO2: 106 [66, 160] vs 176 [104, 252]; p < 0.001) and more frequent nonrespiratory organ failure (nonrespiratory Sequential Organ Failure Assessment score: 6 [4, 9] vs 5 [3, 7]; p = 0.002), thus required more frequently invasive mechanical ventilation (85.2% vs 73.0%; p < 0.001), oxygen rescue therapies (extracorporeal membrane oxygenation 5.8% vs 0.9%; p = 0.003), vasopressor support (79.4% vs 55.0%; p < 0.001), and renal replacement therapy (48.8% vs 22.1%; p < 0.001). After adjustment for potential confounding factors, Middle East respiratory syndrome was independently associated with death compared to non-Middle East respiratory syndrome severe acute respiratory

  7. Immunological aspects of the complex regional pain syndrome (CRPS).

    Science.gov (United States)

    Krämer, Heidrun H

    2012-01-01

    Limb trauma can lead to the development of a complex regional pain syndrome (CRPS). CRPS is a descriptive term of a variety of different symptoms. According to the current IASP-approved criteria, human CRPS can be diagnosed if a combination of signs is present: continuing pain and hyperalgesia, disproportionate to the initial trauma, skin temperature and colour asymmetry, sweating asymmetry, edema, decreased range of motion, and trophic changes. The diagnosis and treatment of human CRPS can be demanding and the pathophysiology underlying the disease is still under investigation. Immunological aspects are considered to play an important role in the development of CRPS. The impact of elevated pro-inflammatory cytokines systemically as well as locally, increased neurogenic inflammation and auto-antibodies in the pathophysiological development of CRPS are discussed in this review.

  8. [Prevention and management of refeeding syndrome in patients with chronic critical illness].

    Science.gov (United States)

    Chen, Jun; Fan, Chaogang

    2016-07-01

    Nutritional support is an important means to treat the patients with chronic critical illness for commonly associated malnutrition. Refeeding syndrome is a serious complication during the process, mainly manifested as severe electrolyte with hypophosphataemia being the most common. Refeeding syndrome is not uncommon but it is often ignored. In our future clinical work, we need to recognize this chinical situation and use preventative and treatment measures. According to NICE clinical nutrition guideline, we discussed the risk factors, treatment methods and preventive measures of refeeding syndrome in patients with chronic critical illness. We argued that for patients with high risk refeeding syndrome, nutritional support treatment should be initially low calorie and slowly increased to complete requirement. Circulation capacity should be recovered, fluid balance must be closely monitored and supplement of vitamins, microelement, electrolytes should be noted. After the emergence of refeeding syndrome, we should reduce or even stop the calorie intake, give an active treatment for electrolyte disorder, provide vitamin B, and maintain the functions of multiple organs.

  9. Syndrome or Difference: A Critical Review of Medical Conceptualisations of Asperger's Syndrome

    Science.gov (United States)

    Wheeler, Marc

    2011-01-01

    We live in a world of vast social differences. People of different ages, cultures, religions, gender and sexual orientation have, and indeed are expected to have, distinct differences in their behaviour, and in the way that they see the world. Asperger's syndrome (AS) is no different. People with AS have different personality types and have…

  10. Complex Regional Pain Syndrome (CRPS Type II After Carpal Tunnel Release Surgery: Case Report

    Directory of Open Access Journals (Sweden)

    Hakan Tunç

    2010-08-01

    Full Text Available Summary Complex regional pain syndrome is a chronic syndrome characterised with dystrophic changes and neurovascular disordes of bone and skin of extremities. The most common etiological factors are trauma, ischemic heart disease, cerebral lesions, servical region disorders, infections, and surgical treatments. Carpal tunnel syndrome is the most common compressive neuropaty of the upper extremity. There are various surgical and conservative alternatives in the treatment of carpal tunnel syndrome. Complex regional pain syndrome has been reported as a complication of surgical carpal tunnel release in 2-5% of patients. In this case report clinical characteristics and rehabilitation outcomes of a patient with complex regional pain syndrome after carpal tunnel release surgery is presented. (Osteoporoz Dünyasından 2010;16:41-3

  11. Regional outbreak of staphylococcal scalded skin syndrome in healthy children

    Directory of Open Access Journals (Sweden)

    Hyun Jeong Do

    2010-01-01

    Full Text Available Purpose : Staphylococcal scalded skin syndrome (SSSS is a relatively uncommon superficial blistering skin disease that is due to Staphylococcus aureus. We had experienced a regional outbreak of SSSS over 3 years in healthy children. Methods : We retrospectively reviewed the medical records of those patients diagnosed as SSSS. Most of neonatal cases were nosocomial infections and excluded from the analysis. The clinical features, laboratory findings, the isolation and antibiotic resistance of S. aureus, the antibiotic management and other supportive treatments were analyzed. Results : Fifty-five patients with SSSS were admitted to our hospital from October 2001 to September 2004. The median age of patients was 3.0 years. Of the 55 patients, 9 were the generalized type, 13 were the intermediate type and 33 were the scarletiniform rash. All the patients were living in neighborhood of the Jinju area. S. aureus were isolated from 9 of the patients and all of the isolated S. aureus were methicillin resistant. All the patients except two were treated with intravenous flocloxacillin or nafcillin and/or cefotaxime. All the patients recovered during the follow-up period of 2 to 3 weeks. Conclusion : We experienced a regional outbreak of SSSS in previous healthy children. Further study for finding the carriers of S. aureus caused SSSS and preventing the spread of this disease is needed. Additionally, guidelines for treating SSSS due to methicillin resistant S. aureus should be established.

  12. Orofacial complex regional pain syndrome: pathophysiologic mechanisms and functional MRI.

    Science.gov (United States)

    Lee, Yeon-Hee; Lee, Kyung Mi; Kim, Hyug-Gi; Kang, Soo-Kyung; Auh, Q-Schick; Hong, Jyung-Pyo; Chun, Yang-Hyun

    2017-08-01

    Complex regional pain syndrome (CRPS) is one of the most challenging chronic pain conditions and is characterized by burning pain, allodynia, hyperalgesia, autonomic changes, trophic changes, edema, and functional loss involving mainly the extremities. Until recently, very few reports have been published concerning CRPS involving the orofacial area. We report on a 50-year-old female patient who presented with unbearable pain in all of her teeth and hypersensitivity of the facial skin. She also reported intractable pain in both extremities accompanied by temperature changes and orofacial pain that increased when the other pains were aggravated. In the case of CRPS with trigeminal neuropathic pain, protocols for proper diagnosis and prompt treatment have yet to be established in academia or in the clinical field. We performed functional magnetic resonance imaging for a thorough analysis of the cortical representation of the affected orofacial area immediately before and immediately after isolated light stimulus of the affected hand and foot and concluded that CRPS can be correlated with trigeminal neuropathy in the orofacial area. Furthermore, the patient was treated with carbamazepine administration and stellate ganglion block, which can result in a rapid improvement of pain in the trigeminal region. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Metabolic syndrome in the Mediterranean region: Current status

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    Panagiotis Anagnostis

    2012-01-01

    Full Text Available Metabolic syndrome (MetS is a cluster of metabolic abnormalities including abdominal obesity, impaired fasting glucose, hypertension and dyslipidemia. It seems to affect about one-fourth to one-fifth of the Mediterranean population, and its prevalence increases with age, being similar for both sexes and depending on the region and the definition used, with the National Cholesterol Education Program-Adult Treatment Panel-III (NCEP-ATPIII definition being the most effective in the identification of glucose intolerance and cardiovascular risk. Except for these, MetS is associated with fatty liver disease, some forms of cancer, hypogonadism, and vascular dementia. The Mediterranean diet seems to be an ideal diet in patients with MetS, being rich in fibre, monounsaturated and polyunsaturated fats, and low in animal protein; and decreases the prevalence of MetS and cardiovascular disease risk. Except for weight loss, multifactorial intervention including insulin resistance reduction and normoglycemia, management of dyslipidemia, optimizing blood pressure and administration of low-dose aspirin for patients at high or moderately high cardiovascular disease (CVD risk are additional targets. The present review provides current understanding about MetS in the Mediterranean region, focusing on its prevalence, clinical significance, and therapeutic strategy.

  14. Metabolic syndrome in the Mediterranean region: Current status.

    Science.gov (United States)

    Anagnostis, Panagiotis

    2012-01-01

    Metabolic syndrome (MetS) is a cluster of metabolic abnormalities including abdominal obesity, impaired fasting glucose, hypertension and dyslipidemia. It seems to affect about one-fourth to one-fifth of the Mediterranean population, and its prevalence increases with age, being similar for both sexes and depending on the region and the definition used, with the National Cholesterol Education Program-Adult Treatment Panel-III (NCEP-ATPIII) definition being the most effective in the identification of glucose intolerance and cardiovascular risk. Except for these, MetS is associated with fatty liver disease, some forms of cancer, hypogonadism, and vascular dementia. The Mediterranean diet seems to be an ideal diet in patients with MetS, being rich in fibre, monounsaturated and polyunsaturated fats, and low in animal protein; and decreases the prevalence of MetS and cardiovascular disease risk. Except for weight loss, multifactorial intervention including insulin resistance reduction and normoglycemia, management of dyslipidemia, optimizing blood pressure and administration of low-dose aspirin for patients at high or moderately high cardiovascular disease (CVD) risk are additional targets. The present review provides current understanding about MetS in the Mediterranean region, focusing on its prevalence, clinical significance, and therapeutic strategy.

  15. Evidence based guidelines for complex regional pain syndrome type 1

    Directory of Open Access Journals (Sweden)

    Thomassen-Hilgersom Ilona L

    2010-03-01

    Full Text Available Abstract Background Treatment of complex regional pain syndrome type I (CRPS-I is subject to discussion. The purpose of this study was to develop multidisciplinary guidelines for treatment of CRPS-I. Method A multidisciplinary task force graded literature evaluating treatment effects for CRPS-I according to their strength of evidence, published between 1980 to June 2005. Treatment recommendations based on the literature findings were formulated and formally approved by all Dutch professional associations involved in CRPS-I treatment. Results For pain treatment, the WHO analgesic ladder is advised with the exception of strong opioids. For neuropathic pain, anticonvulsants and tricyclic antidepressants may be considered. For inflammatory symptoms, free-radical scavengers (dimethylsulphoxide or acetylcysteine are advised. To promote peripheral blood flow, vasodilatory medication may be considered. Percutaneous sympathetic blockades may be used to increase blood flow in case vasodilatory medication has insufficient effect. To decrease functional limitations, standardised physiotherapy and occupational therapy are advised. To prevent the occurrence of CRPS-I after wrist fractures, vitamin C is recommended. Adequate perioperative analgesia, limitation of operating time, limited use of tourniquet, and use of regional anaesthetic techniques are recommended for secondary prevention of CRPS-I. Conclusions Based on the literature identified and the extent of evidence found for therapeutic interventions for CRPS-I, we conclude that further research is needed into each of the therapeutic modalities discussed in the guidelines.

  16. Genome-wide expression profiling of complex regional pain syndrome.

    Directory of Open Access Journals (Sweden)

    Eun-Heui Jin

    Full Text Available Complex regional pain syndrome (CRPS is a chronic, progressive, and devastating pain syndrome characterized by spontaneous pain, hyperalgesia, allodynia, altered skin temperature, and motor dysfunction. Although previous gene expression profiling studies have been conducted in animal pain models, there genome-wide expression profiling in the whole blood of CRPS patients has not been reported yet. Here, we successfully identified certain pain-related genes through genome-wide expression profiling in the blood from CRPS patients. We found that 80 genes were differentially expressed between 4 CRPS patients (2 CRPS I and 2 CRPS II and 5 controls (cut-off value: 1.5-fold change and p<0.05. Most of those genes were associated with signal transduction, developmental processes, cell structure and motility, and immunity and defense. The expression levels of major histocompatibility complex class I A subtype (HLA-A29.1, matrix metalloproteinase 9 (MMP9, alanine aminopeptidase N (ANPEP, l-histidine decarboxylase (HDC, granulocyte colony-stimulating factor 3 receptor (G-CSF3R, and signal transducer and activator of transcription 3 (STAT3 genes selected from the microarray were confirmed in 24 CRPS patients and 18 controls by quantitative reverse transcription-polymerase chain reaction (qRT-PCR. We focused on the MMP9 gene that, by qRT-PCR, showed a statistically significant difference in expression in CRPS patients compared to controls with the highest relative fold change (4.0±1.23 times and p = 1.4×10(-4. The up-regulation of MMP9 gene in the blood may be related to the pain progression in CRPS patients. Our findings, which offer a valuable contribution to the understanding of the differential gene expression in CRPS may help in the understanding of the pathophysiology of CRPS pain progression.

  17. Motor imagery and its effect on complex regional pain syndrome: an integrative review

    Directory of Open Access Journals (Sweden)

    Nélio Silva de Souza

    2015-12-01

    Full Text Available The motor imagery (MI has been proposed as a treatment in the complex regional pain syndrome type 1 (CRPS-1, since it seems to promote a brain reorganization effect on sensory- motor areas of pain perception. The aim of this paper is to investigate, through an integrative critical review, the influence of MI on the CRPS-1, correlating their evidence to clinical practice. Research in PEDro, Medline, Bireme and Google Scholar databases was conducted. Nine randomized controlled trials (level 2, 1 non-controlled clinical study (level 3, 1 case study (level 4, 1 systematic review (level 1, 2 review articles and 1 comment (level 5 were found. We can conclude that MI has shown effect in reducing pain and functionality that remains after 6 months of treatment. However, the difference between the MI strategies for CRPS-1 is unknown as well as the intensity of mental stress influences the painful response or effect of MI or other peripheral neuropathies.

  18. Thermodynamic properties of water in the critical region

    International Nuclear Information System (INIS)

    Veloso, Marcelo A.

    2009-01-01

    The supercritical-water-cooled reactor (SCWR) is one of the nuclear reactor technologies selected for research and development under the Generation IV program. SCWRs offer the potential for high thermal efficiencies and considerable plant simplifications for improved economics. One of the main characteristics of critical water is the strong variations of its thermal-physical properties in the vicinity of the critical point. These large variations may result in an unusual heat transfer behavior. The 1967 IFC Formulation for Industrial Use, which until 1998 formed the basis of steam tables used in many areas of steam power industry throughout the world since the late 1960's, has been now replaced with the IAPWS IF-97 Formulation for the Thermodynamic Properties of Water and Steam for Industrial Use, adopted by the International Association for the Properties of Water and Steam (IAPWS) in 1997. An IAPWS release points out that this new formulation has some unsatisfactory features in the immediate vicinity of the critical point. In order to investigate this singular aspect, which is crucial to better understand the heat transfer mechanism in a SCWR system, predictions by the IAPWS-IF97 formulation will be compared with thermodynamic properties values predicted by an alternative crossover equation of state as well as with experimental data found in literature. (author)

  19. Complex regional pain syndrome 1 – the Swiss cohort study

    Directory of Open Access Journals (Sweden)

    Perez Roberto SGM

    2008-06-01

    Full Text Available Abstract Background Little is known about the course of Complex Regional Pain Syndrome 1 and potential factors influencing the course of this disorder over time. The goal of this study is a to set up a database with patients suffering from suspected CRPS 1 in an initial stadium, b to perform investigations on epidemiology, diagnosis, prognosis, and socioeconomics within the database and c to develop a prognostic risk assessment tool for patients with CRPS 1 taking into account symptomatology and specific therapies. Methods/design Prospective cohort study. Patients suffering from a painful swelling of the hand or foot which appeared within 8 weeks after a trauma or a surgery and which cannot be explained by conditions that would otherwise account for the degree of pain and dysfunction will be included. In accordance with the recommendations of International Classification of Functioning, Disability and Health (ICF model, standardised and validated questionnaires will be used. Patients will be monitored over a period of 2 years at 6 scheduled visits (0 and 6 weeks, 3, 6, 12, and 24 months. Each visit involves a physical examination, registration of therapeutic interventions, and completion of the various study questionnaires. Outcomes involve changes in health status, quality of life and costs/utility. Discussion This paper describes the rationale and design of patients with CRPS 1. Ideally, potential risk factors may be identified at an early stage in order to initiate an early and adequate treatment in patients with increased risk for delayed recovery. Trial registration Not applicable

  20. Activation of cutaneous immune responses in complex regional pain syndrome

    Science.gov (United States)

    Birklein, Frank; Drummond, Peter D.; Li, Wenwu; Schlereth, Tanja; Albrecht, Nahid; Finch, Philip M.; Dawson, Linda F.; Clark, J. David; Kingery, Wade S.

    2014-01-01

    The pathogenesis of complex regional pain syndrome (CRPS) is unresolved, but TNF-α and IL-6 are elevated in experimental skin blister fluid from CRPS affected limbs, as is tryptase, a marker for mast cells. In the rat fracture model of CRPS exaggerated sensory and sympathetic neural signaling stimulate keratinocyte and mast cell proliferation, causing the local production of high levels of inflammatory cytokines leading to pain behavior. The current investigation used CRPS patient skin biopsies to determine whether keratinocyte and mast cell proliferation occur in CRPS skin and to identify the cellular source of the up-regulated TNF-α, IL-6, and tryptase observed in CRPS experimental skin blister fluid. Skin biopsies were collected from the affected skin and the contralateral mirror site in 55 CRPS patients and the biopsy sections were immunostained for keratinocyte, cell proliferation, mast cell markers, TNF-α, and IL-6. In early CRPS keratinocytes were activated in the affected skin, resulting in proliferation, epidermal thickening, and up-regulated TNF-α and IL-6 expression. In chronic CRPS there was reduced keratinocyte proliferation with epidermal thinning in the affected skin. Acute CRPS patients also had increased mast cell accumulation in the affected skin, but there was no increase in mast cell numbers in chronic CRPS. PMID:24462502

  1. Complex regional pain syndromes (CRPS type 1 validating case histories

    Directory of Open Access Journals (Sweden)

    P. Berger

    2003-01-01

    Full Text Available The treatment of patients with complex regional pain syndrome (CRPS type 1 is challenging and unpredictable as the condition presents with vascular and neuropathic symptoms after nil or even minor injury to a peripheral nerve. The condition is one of a pain and motor dysfunction. The pathophysiology is not well understood and the relief of symptoms may change from being sympathetically mediated to sympathetically independent during  the course of the disease. At any stage physiotherapy has been advocated as the corner stone and most important aspect of treatment in the rehabilitation of these individuals but unfortunately it has been difficult to execute when pain is exacerbated due to allodynia (unbearable to touch or move and hyperalgesia. Best results have been obtained if the patients are recognised and treated in the early or acute phase and it has been found that through careful assessment and analysis these patients can be recognised by previous events that have occurred in their initial case history. The treatment in the acute stage with physiotherapy modalities such as electrical stimulation and acupuncture will produce an early cessation of the symptoms and prevention of the disease developing into the fully blown CRPS type 1 with irreversible and possibly atrophic consequences. Case histories have been presented that illustrate these important aspects and demonstrate  the value of early and the appropriate physiotherapy that may be more successful than other pharmacological and physical interventions in this disease.

  2. Reflex sympathetic dystrophy/complex regional pain syndrome, type 1

    Directory of Open Access Journals (Sweden)

    S.H. Botha

    2004-06-01

    Full Text Available Complex regional pain syndrome (CPRS, type 1 is a pain disorder that develops unpredictably and can follow a minor injury. A 12-year-old boy presented with severe pain in the feet and could not walk or stand weight bearing. Normal X-rays showed osteopenic changes and radiolucent lines, which appeared to be stress fractures. Three-phase bone scintigraphy showed no uptake in the left lower leg on the blood pool phase or on the immediate or delayed images. This indicated typical CPRS type 1 in children. The uptake in the right foot was increased and the stress fracture and other illness could not be differentiated. Computed tomography was done to exclude stress fractures. Only osteopenic changes in both calcaneus bones were found and there was no evidence of cortical stress fractures. Magnetic resonance images revealed oedema in the calcaneus and talus bones of both feet. The patient received epidural narcotic infusion with sympathetic blockage for 1 week combined with extensive physiotherapy. The blood pool phase of the bone scan became normal within 2 weeks, and increased uptake in both feet was noticed. The patient was followed up with MRI every 3 months and the bone marrow oedema disappeared after 6 months.

  3. Complex Regional Pain Syndrome after Transradial Cardiac Catheterization

    Directory of Open Access Journals (Sweden)

    Chih-Jou Lai

    2006-04-01

    Full Text Available Complex regional pain syndrome (CRPS is a disease with unclear pathophysiology. The condition is characterized by pain, soft tissue change, vasomotor change, and even psychosocial disturbance. It may affect the upper more than the lower extremities, and the distal more than the proximal. The trigger factors include carpal tunnel release, Dupuytren's repair, tendon release procedures, knee surgery, crush injury, ankle arthrodesis, amputation, and hip arthroplasty. Rarely, it has been associated with stroke, mastectomy, pregnancy, and osteogenesis imperfecta. Herein, we present a rare case of a patient who was diagnosed with CRPS after transradial cardiac catheterization. CRPS was first diagnosed due to hand swelling, allodynia, paresthesia, and the limited range of motion of interphalangeal, metacarpophalangeal, and wrist joints, with the preceding factor of transradial cardiac catheterization, and was then confirmed by a three-phase bone scan. After intensive physical therapy with hydrotherapy, manual soft tissue release, and occupational therapy for the hand function, there was much improvement in range of motion and hand function. There was no allodynia or painful sensation in the follow-up. After training, the functional status of this patient was adequate for daily activity.

  4. Regional innovation systems in Portugal: a critical evaluation

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    Domingos Santos

    2014-01-01

    Full Text Available La innovación ha pasado a primer plano en la política regional en las tres últimas décadas. Las políticas públicas han sido diseñadas por los «modelos de mejores prácticas» derivadas de las zonas urbano-metropolitanas de alta tecnología y regiones exitosas. Sin embargo, las lecciones aprendidas de estos ejemplos son raramente transferibles a otras partes. Los sistemas regionales de innovación en las regiones periféricas, y la posibilidad de su actuación como instrumentos de competitividad territorial, rara vez han sido objeto de discusión. El objetivo principal del artículo es, precisamente, tener a Portugal como un ejemplo para enriquecer este análisis. En la primera parte de este artículo se examina el concepto de sistemas de innovación regional en el contexto de las modernas teorías de la innovación y de las políticas regionales. Se argumenta que el papel del aprendizaje localizado es de importancia estratégica en la promoción del desarrollo regional endógeno. Luego, los autores discuten las barreras estructurales y oportunidades para promover estrategias regionales de innovación en el contexto político, económico y social portugués, y, por último, se señalan algunas especificidades que deben ser abordadas en el rediseño de las intervenciones públicas con el fin de mejorar la competitividad regional y la sostenibilidad.

  5. Critical issues of alcohol safety in the region

    OpenAIRE

    Svetlana Vasil’evna Aksyutina; Natal’ya Mikhailovna Ovsyankina

    2015-01-01

    The paper presents results of the research into the economic and socio-demographic indicators associated with the production and consumption of alcoholic beverages. It discloses the analysis of the alcoholic beverage market structure in the Vologda Oblast. The authors have identified the threshold of the safe alcohol production volume in the region taking into account the World Health Organization standards of alcohol consumption and the share of illegally produced goods. The article states t...

  6. Comparing critical success factors for PV between three regions

    International Nuclear Information System (INIS)

    Groenendaal, B.J.; Roosmalen, J.A.M. van

    2000-01-01

    As a research method the project team has chosen to survey the opinion of PV experts and persons involved in the implementation of PV. Therefore, a questionnaire was sent to about 300 persons spread among 3 regions America, Europe and Asia. The returned questionnaires have been statistical analysed with the software tool SPSS. The used analytical methods can be divided in a comparing method (Mann-Whitney test) and ranking methods (Friedman test and the medal classification test). General conclusions are that there is a significant difference in answers between America and Europe about the significance of 'cost reduction'. Also Asia is significant different from America and Europe for the factor 'technical' reliability. There is a significant difference about the status of financing between America and Europe versus Asia. All the regions have a different opinion about the status of RD and D'. Also the status of 'the PV network' is significant different between Asia and America. America and Europe rank 'financial aspects' and 'cost reduction' as the most significant aspects while Asia rank 'specialist knowledge' and 'the PV network' as the most significant. All regions rank 'specialist knowledge' having the worst status and rank 'environmental merits' and 'technical reliability' having the best status. Finally Europe and Asia differ significant about which aspect is the most important for the factors 'internationalisation and other activities'. Europe finds 'harmonisation of policy' the most important internationalisation aspect and Asia prefers 'development aid'. Asia prefers 'pioneering activities' and Europe prefers 'initiatives by social organisations' as most important other activity. (au)

  7. Secondary late-onset Lennox-Gastaut syndrome: a critical view

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    Amilton Antunes Barreira

    1984-06-01

    Full Text Available From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxismal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or beter neu-ropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatolgy. The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.

  8. Estrogens and the risk of complex regional pain syndrome (CRPS).

    Science.gov (United States)

    de Mos, M; Huygen, F J P M; Stricker, B H Ch; Dieleman, J P; Sturkenboom, M C J M

    2009-01-01

    Since complex regional pain syndrome (CRPS) shows a clear female predominance, we investigated the association between the cumulative as well as current exposure to estrogens, and CRPS. A population-based case-control study was conducted in the Integrated Primary Care Information (IPCI) project in the Netherlands. Cases were identified from electronic records (1996-2005) and included if they were confirmed during a visit (using International Association for the Study of Pain Criteria), or had been diagnosed by a specialist. Controls were matched to cases on gender, age, calendar time, and injury. Measures of cumulative endogenous estrogen exposure were obtained by questionnaire and included age of menarche and menopause, menstrual life, and cumulative months of pregnancy and breast-feeding. Current estrogen exposure at CRPS onset was retrieved from the electronic medical records and determined by current pregnancy or by the use of oral contraceptive (OC) drugs or hormonal replacement therapy (HRT). Hundred and forty-three female cases (1493 controls) were included in analyses on drug use and pregnancies, while cumulative endogenous estrogen exposure was studied in 53 cases (58 controls) for whom questionnaire data were available. There was no association between CRPS and either cumulative endogenous estrogen exposure, OC, or HRT use. CRPS onset was increased during the first 6 months after pregnancy (OR: 5.6, 95%CI: 1.0-32.4), although based on small numbers. We did not find an association between CRPS onset and cumulative endogenous estrogen exposure or current OC or HRT use, but more powered studies are needed to exclude potential minor associations.

  9. Sensitization of the Nociceptive System in Complex Regional Pain Syndrome

    Science.gov (United States)

    Diedrichs, Carolina; Baron, Ralf; Gierthmühlen, Janne

    2016-01-01

    Background Complex regional pain syndrome type I (CRPS-I) is characterized by sensory, motor and autonomic abnormalities without electrophysiological evidence of a nerve lesion. Objective Aims were to investigate how sensory, autonomic and motor function change in the course of the disease. Methods 19 CRPS-I patients (17 with acute, 2 with chronic CRPS, mean duration of disease 5.7±8.3, range 1–33 months) were examined with questionnaires (LANSS, NPS, MPI, Quick DASH, multiple choice list of descriptors for sensory, motor, autonomic symptoms), motor and autonomic tests as well as quantitative sensory testing according to the German Research Network on Neuropathic Pain at two visits (baseline and 36±10.6, range 16–53 months later). Results CRPS-I patients had an improvement of sudomotor and vasomotor function, but still a great impairment of sensory and motor function upon follow-up. Although pain and mechanical detection improved upon follow-up, thermal and mechanical pain sensitivity increased, including the contralateral side. Increase in mechanical pain sensitivity and loss of mechanical detection were associated with presence of ongoing pain. Conclusions The results demonstrate that patients with CRPS-I show a sensitization of the nociceptive system in the course of the disease, for which ongoing pain seems to be the most important trigger. They further suggest that measured loss of function in CRPS-I is due to pain-induced hypoesthesia rather than a minimal nerve lesion. In conclusion, this article gives evidence for a pronociceptive pain modulation profile developing in the course of CRPS and thus helps to assess underlying mechanisms of CRPS that contribute to the maintenance of patients’ pain and disability. PMID:27149519

  10. Sensitization of the Nociceptive System in Complex Regional Pain Syndrome.

    Directory of Open Access Journals (Sweden)

    Maren Reimer

    Full Text Available Complex regional pain syndrome type I (CRPS-I is characterized by sensory, motor and autonomic abnormalities without electrophysiological evidence of a nerve lesion.Aims were to investigate how sensory, autonomic and motor function change in the course of the disease.19 CRPS-I patients (17 with acute, 2 with chronic CRPS, mean duration of disease 5.7±8.3, range 1-33 months were examined with questionnaires (LANSS, NPS, MPI, Quick DASH, multiple choice list of descriptors for sensory, motor, autonomic symptoms, motor and autonomic tests as well as quantitative sensory testing according to the German Research Network on Neuropathic Pain at two visits (baseline and 36±10.6, range 16-53 months later.CRPS-I patients had an improvement of sudomotor and vasomotor function, but still a great impairment of sensory and motor function upon follow-up. Although pain and mechanical detection improved upon follow-up, thermal and mechanical pain sensitivity increased, including the contralateral side. Increase in mechanical pain sensitivity and loss of mechanical detection were associated with presence of ongoing pain.The results demonstrate that patients with CRPS-I show a sensitization of the nociceptive system in the course of the disease, for which ongoing pain seems to be the most important trigger. They further suggest that measured loss of function in CRPS-I is due to pain-induced hypoesthesia rather than a minimal nerve lesion. In conclusion, this article gives evidence for a pronociceptive pain modulation profile developing in the course of CRPS and thus helps to assess underlying mechanisms of CRPS that contribute to the maintenance of patients' pain and disability.

  11. Complex regional pain syndrome 1 : a study on pain and motor impairments

    NARCIS (Netherlands)

    G.M. Ribbers (Gerard)

    2001-01-01

    textabstractThis thesis, is compiled of publications on reflex sympathetic dystrophy (RSD) or Complex Regional Pain Syndrome type 1 (CRPS 1), as it was renamed by the committee on taxonomy of the International Association for the Study of Pain (IASP). It is a puzzling syndrome both from a clinical

  12. Aggregation propensity of critical regions of the protein Tau

    Science.gov (United States)

    Muthee, Micaiah; Ahmed, Azka; Larini, Luca

    The Alzheimer's disease is an irreversible, progressive brain disorder that slowly destroys memory and thinking skills, which eventually leads to the ability to not able to carry out the simplest tasks. The Alzheimer's disease is characterized by the formation of protein aggregates both within and outside of the brain's cells, the neurons. Within the neurons, the aggregation of the protein tau leads to the destruction of the microtubules in the axon of the neuron. Tau belongs to a group of proteins referred to as Microtubule-Associated Proteins. It is extremely flexible and is classified as an intrinsically unstructured protein due to its low propensity to form secondary structure. Tau promotes tubulin assembly into microtubules thereby stabilizing the cytoskeleton of the axon of the neurons. The microtubule binding region of tau consists of 4 pseudo-repeats. In this study, we will focus on the aggregation propensity of two fragments. In this study we will focus on the PHF43 fragment that contains the third pseudo-repeat and has been shown experimentally to aggregate readily. Another fragment that contains the second pseudo-repeat will be considered as well. Mutations in this region are associated with various form of dementia and for this reason we will consider the mutant P301L.

  13. Critical Hydrologic and Atmospheric Measurements in Complex Alpine Regions

    Science.gov (United States)

    Parlange, M. B.; Bou-Zeid, E.; Barrenetxea, G.; Krichane, M.; Ingelrest, F.; Couach, O.; Luyet, V.; Vetterli, M.; Lehning, M.; Duffy, C.; Tobin, C.; Selker, J.; Kumar, M.

    2007-12-01

    The Alps are often referred to as the « Water Towers of Europe » and as such play an essential role in European water resources. The impact of climatic change is expected to be particularly pronounced in the Alps and the lack of detailed hydrologic field observations is problematic for predictions of hydrologic and hazard assessment. Advances in information technology and communications provide important possibilities to improve the situation with relatively few measurements. We will present sensorscope technology (arrays of wireless weather stations including soil moisture, pressure, and temperature) that has now been deployed at the Le Genepi and Grand St. Bernard pass. In addition, a Distributed Temperature Sensor array on the stream beds has been deployed and stream discharge monitored. The high spatial resolution data collected in these previously "ungaged" regions are used in conjunction with new generation hydrologic models. The framework as to what is possible today with sensor arrays and modeling in extreme mountain environments is discussed.

  14. Critical issues of alcohol safety in the region

    Directory of Open Access Journals (Sweden)

    Svetlana Vasil’evna Aksyutina

    2015-03-01

    Full Text Available The paper presents results of the research into the economic and socio-demographic indicators associated with the production and consumption of alcoholic beverages. It discloses the analysis of the alcoholic beverage market structure in the Vologda Oblast. The authors have identified the threshold of the safe alcohol production volume in the region taking into account the World Health Organization standards of alcohol consumption and the share of illegally produced goods. The article states that the increased alcohol production contributes to the rise in tax revenues, but the state fiscal policy to regulate the alcoholic beverage market leads to an increase in the share of shadow turnover. The authors have calculated the economic loss connected with the illegal production of alcoholic beverages in the Vologda Oblast. The alcohol consumption is a destructive socio-demographic process and one of the threats to the health of the nation. Excessive alcohol consumption leads to alcohol dependence, regression of the society and increases the threat to national and economic security. The study reveals a direct correlation between the consumption of alcoholic beverages per capita and mortality rates in men and women of working age from the causes related to the consumption of alcoholic beverages. The study of the international experience to regulate alcohol consumption has showed the need to tighten state control in the sphere of production and turnover of alcoholic products. The conduct of the unified state alcohol policy substantiates the selection of the alcohol industry in the all-Russian classifier of economic activity types. The authors have elaborated the concept and conditions of alcoholic security from the point of view of economic growth and social development. The article substantiates the necessity to monitor alcohol safety indicators when considering the regional development. It presents the complex system of socio-economic and demographic

  15. AN AUDIT OF THE SUDDEN-INFANT-DEATH-SYNDROME PREVENTION PROGRAM IN THE AUCKLAND REGION

    NARCIS (Netherlands)

    Obdeijn, M. C.; Tonkin, S.; Mitchell, E. A.

    1995-01-01

    Aim. An audit of the sudden infant death syndrome (SIDS) prevention programme in the Auckland region. Methods. 107 health professionals working in antenatal classes, postnatal wards, domiciliary midwifery and the Plunket Society were interviewed. Results. Maternal smoking and infant sleeping

  16. Scapulothoracic bursitis and snapping scapula syndrome: a critical review of current evidence.

    Science.gov (United States)

    Warth, Ryan J; Spiegl, Ulrich J; Millett, Peter J

    2015-01-01

    Symptomatic scapulothoracic disorders, such as painful scapular crepitus and/or bursitis, are uncommon; however, they can produce significant pain and disability in many patients. To review the current knowledge pertaining to snapping scapula syndrome and to identify areas of further research that may be helpful to improve clinical outcomes and patient satisfaction. Systematic review. We performed a preliminary search of the PubMed and Embase databases using the search terms "snapping scapula," "scapulothoracic bursitis," "partial scapulectomy," and "superomedial angle resection" in September 2013. All nonreview articles related to the topic of snapping scapula syndrome were included. The search identified a total of 167 unique articles, 81 of which were relevant to the topic of snapping scapula syndrome. There were 36 case series of fewer than 10 patients, 16 technique papers, 11 imaging studies, 9 anatomic studies, and 9 level IV outcomes studies. The level of evidence obtained from this literature search was inadequate to perform a formal systematic review or meta-analysis. Therefore, a critical review of current evidence is presented. Snapping scapula syndrome, a likely underdiagnosed condition, can produce significant shoulder dysfunction in many patients. Because the precise origin is typically unknown, specific treatments that are effective for some patients may not be effective for others. Nevertheless, bursectomy with or without partial scapulectomy is currently the most effective primary method of treatment in patients who fail nonoperative therapy. However, many patients experience continued shoulder disability even after surgical intervention. Future studies should focus on identifying the modifiable factors associated with poor outcomes after operative and nonoperative management for snapping scapula syndrome in an effort to improve clinical outcomes and patient satisfaction. © 2014 The Author(s).

  17. Effects of levosimendan for low cardiac output syndrome in critically ill patients

    DEFF Research Database (Denmark)

    Koster, Geert; Wetterslev, Jørn; Gluud, Christian

    2015-01-01

    PURPOSE: To assess the benefits and harms of levosimendan for low cardiac output syndrome in critically ill patients. METHODS: We conducted a systematic review with meta-analyses and trial sequential analyses (TSA) of randomised clinical trials comparing levosimendan with any type of control. Two...... in the systematic review and 49 trials (6,688 patients) in the meta-analysis. One trial had low risk of bias and nine trials (2,490 patients) were considered lower risk of bias. Trials compared levosimendan with placebo, control interventions, and other inotropes. Pooling all trials including heterogenous...

  18. MicroRNA modulation in complex regional pain syndrome

    Directory of Open Access Journals (Sweden)

    Orlova Irina A

    2011-11-01

    Full Text Available Abstract Background Aberrant expression of small noncoding RNAs called microRNAs (miRNAs is a common feature of several human diseases. The objective of the study was to identify miRNA modulation in patients with complex regional pain syndrome (CRPS a chronic pain condition resulting from dysfunction in the central and/or peripheral nervous systems. Due to a multitude of inciting pathologies, symptoms and treatment conditions, the CRPS patient population is very heterogeneous. Our goal was to identify differentially expressed miRNAs in blood and explore their utility in patient stratification. Methods We profiled miRNAs in whole blood from 41 patients with CRPS and 20 controls using TaqMan low density array cards. Since neurogenic inflammation is known to play a significant role in CRPS we measured inflammatory markers including chemokines, cytokines, and their soluble receptors in blood from the same individuals. Correlation analyses were performed for miRNAs, inflammatory markers and other parameters including disease symptoms, medication, and comorbid conditions. Results Three different groups emerged from miRNA profiling. One group was comprised of 60% of CRPS patients and contained no control subjects. miRNA profiles from the remaining patients were interspersed among control samples in the other two groups. We identified differential expression of 18 miRNAs in CRPS patients. Analysis of inflammatory markers showed that vascular endothelial growth factor (VEGF, interleukin1 receptor antagonist (IL1Ra and monocyte chemotactic protein-1 (MCP1 were significantly elevated in CRPS patients. VEGF and IL1Ra showed significant correlation with the patients reported pain levels. Analysis of the patients who were clustered according to their miRNA profile revealed correlations that were not significant in the total patient population. Correlation analysis of miRNAs detected in blood with additional parameters identified miRNAs associated with

  19. Demographic Features in Patients with Complex Regional Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Berat Meryem Alkan

    2011-12-01

    Full Text Available Summary Özet Orijinal Araştırma / Original Investigation 77 Aim: Complex regional pain syndrome (CRPS is characterized by pain, vasomotor and sudomotor changes and trophic disturbances. It may develop as a result of trauma, especially extremity fractures and surgery. Although the pathogenesis of CRPS is not exactly clear explained, it has been suggested that central and periferal mechanisms play role and neurogenic inflamatuar and microvasculer functional impairmensts are accompanying to the process. Not all but some of the patients with past trauma or with other possible etiological factors develop CRPS. This suggests the presence of an individual predisposition. In this article, we searched post fractüred CRPS-I patients demographic characteristics, current systemic diseases and symptoms that accompany a CRPS in our records. Materials and Methods: In this retrospective study conducted in Ankara Atatürk Education and Research Hospital, medical records of 356 patients admitted to physical medicine and rehabilitation outpatient clinics with fractures between January 2011 and June 2011 were evaluated and 34 patients diagnosed as CRPS-1 were included in the study. Results: 34 of 356 patients (9.56% with fractures were diagnosed as CRPS-1 in our outpatient clinics. Mean age of the patients was 46.05 years and 10 patients were females (29.4% and 24 patients (70.6% were males. Fractures were in upper extremities in 18 patients (52.9% and in lower extremities in 16 (47.1% patients. Neuropsychiatric disorders and other systemic diseases that may have a role in etiology of CRPS were found in lower rates in medical records of our patients. Conclusion: CRPS does not develop in every patients after travma who has etiologic risk factors, so it reminds that there exists a tendency to CRPS. We have observed that CRPS-1 risk was increased in male patients and in upper extremity fractures. We did not observe any other impertant factor which increases

  20. Interpretation of chest radiographs in both cancer and other critical care patients with acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Sema Yilmaz

    2013-04-01

    Full Text Available Acute respiratory distress syndrome is a clinical, pathophysiological and radiographic pattern that has signs of pulmonary edema occur without elevated pulmonary venous pressures. Clinical presentation and progression of acute respiratory distress syndrome are followed by frequently ordered portable chest X-ray in critically ill patients. We evaluated chest radiographs of ten cancer and other six critical care pediatric patients. The parenchymal imaging of lung in patients with cancer was reported the same as that of other critically ill children despite underlying pathophysiological variations in our investigation. [Cukurova Med J 2013; 38(2.000: 270-273

  1. Saturated properties prediction in critical region by a quartic equation of state

    Directory of Open Access Journals (Sweden)

    Yong Wang

    2011-08-01

    Full Text Available A diverse substance library containing extensive PVT data for 77 pure components was used to critically evaluate the performance of a quartic equation of state and other four famous cubic equations of state in critical region. The quartic EOS studied in this work was found to significantly superior to the others in both vapor pressure prediction and saturated volume prediction in vicinity of critical point.

  2. A parametric model for the global thermodynamic behavior of fluids in the critical region

    International Nuclear Information System (INIS)

    Luettmer-Strathmann, J.; Tang, S.; Sengers, J.V.

    1992-01-01

    The asymptotic thermodynamic behavior of fluids near the critical point is described by scaling laws with universal scaling functions that can be represented by parametric equations. In this paper, we derive a more general parametric model that incorporates the crossover from singular thermodynamic behavior near the critical point to regular classical thermodynamic behavior far away from the critical point. Using ethane as an example, we show that such a parametric crossover model yields an accurate representation of the thermodynamic properties of fluids in a large region around the critical point

  3. Role of a critical visceral adipose tissue threshold (CVATT) in metabolic syndrome: implications for controlling dietary carbohydrates: a review

    OpenAIRE

    Freedland Eric S

    2004-01-01

    Abstract There are likely many scenarios and pathways that can lead to metabolic syndrome. This paper reviews mechanisms by which the accumulation of visceral adipose tissue (VAT) may contribute to the metabolic syndrome, and explores the paradigm of a critical VAT threshold (CVATT). Exceeding the CVATT may result in a number of metabolic disturbances such as insulin resistance to glucose uptake by cells. Metabolic profiles of patients with visceral obesity may substantially improve after onl...

  4. Critical pathways of change in fruit export regions at desert margin (Chile)

    DEFF Research Database (Denmark)

    Frederiksen, Peter

    The purpose is to elucidate how critical pathways function in a fruit export region at the desert margin in Chile. The region was investigated at the system level as an open land system with managed fruit plantations in a geographically complex valley. Data collection procedures included total...... change changed pathways. Pathways resulted from a combination of global value chains, the adoption of innovations, past climate change, and regional conditions at different scales. Main pathways of change were upgrade and downgrade of the fruit export region and irrigation systems, whereas the breaking...... areas and not in others. The probable future is expected to be increased separation of intraregional pathways and a more imbalanced region. The conclusion is that openness is the main property responsible for critical pathways of change in the region....

  5. Identifying hotspots and management of critical ecosystem services in rapidly urbanizing Yangtze River Delta Region, China.

    Science.gov (United States)

    Cai, Wenbo; Gibbs, David; Zhang, Lang; Ferrier, Graham; Cai, Yongli

    2017-04-15

    Rapid urbanization has altered many ecosystems, causing a decline in many ecosystem services, generating serious ecological crisis. To cope with these challenges, we presented a comprehensive framework comprising five core steps for identifying and managing hotspots of critical ecosystem services in a rapid urbanizing region. This framework was applied in the case study of the Yangtze River Delta (YRD) Region. The study showed that there was large spatial heterogeneity in the hotspots of ecosystem services in the region, hotspots of supporting services and regulating services aggregately distributing in the southwest mountainous areas while hotspots of provisioning services mainly in the northeast plain, and hotspots of cultural services widespread in the waterbodies and southwest mountainous areas. The regionalization of the critical ecosystem services was made through the hotspot analysis. This study provided valuable information for environmental planning and management in a rapid urbanizing region and helped improve China's ecological redlines policy at regional scale. Copyright © 2017 Elsevier Ltd. All rights reserved.

  6. Candidate region for Coffin-Siris syndrome at 7q32-->34.

    Science.gov (United States)

    McGhee, E M; Klump, C J; Bitts, S M; Cotter, P D; Lammer, E J

    2000-07-31

    Coffin-Siris syndrome is characterized by intrauterine growth retardation, mental deficiency, coarse face, hypoplastic fifth fingers and nails, hirsutism, and initial difficulties with feeding. The etiology of this syndrome is unknown. We report on an 11-year-old girl with Coffin-Siris syndrome and a de novo, apparently balanced reciprocal translocation between chromosomes 7 and 22 [t(7;22)(q32;q11.2)]. The 7q breakpoint in our patient is very similar to the breakpoint reported in a previous case [McPherson et al., 1997: Am J Med Genet 71:430-433] with a balanced t(1;7)(q21.3;q34). Together, these patients provide evidence that the region 7q32-->34 is a candidate region for the gene responsible for Coffin-Siris syndrome.

  7. Differentiating Delirium From Sedative/Hypnotic-Related Iatrogenic Withdrawal Syndrome: Lack of Specificity in Pediatric Critical Care Assessment Tools.

    Science.gov (United States)

    Madden, Kate; Burns, Michele M; Tasker, Robert C

    2017-06-01

    To identify available assessment tools for sedative/hypnotic iatrogenic withdrawal syndrome and delirium in PICU patients, the evidence supporting their use, and describe areas of overlap between the components of these tools and the symptoms of anticholinergic burden in children. Studies were identified using PubMed and EMBASE from the earliest available date until July 3, 2016, using a combination of MeSH terms "delirium," "substance withdrawal syndrome," and key words "opioids," "benzodiazepines," "critical illness," "ICU," and "intensive care." Review article references were also searched. Human studies reporting assessment of delirium or iatrogenic withdrawal syndrome in children 0-18 years undergoing critical care. Non-English language, exclusively adult, and neonatal intensive care studies were excluded. References cataloged by study type, population, and screening process. Iatrogenic withdrawal syndrome and delirium are both prevalent in the PICU population. Commonly used scales for delirium and iatrogenic withdrawal syndrome assess signs and symptoms in the motor, behavior, and state domains, and exhibit considerable overlap. In addition, signs and symptoms of an anticholinergic toxidrome (a risk associated with some common PICU medications) overlap with components of these scales, specifically in motor, cardiovascular, and psychiatric domains. Although important studies have demonstrated apparent high prevalence of iatrogenic withdrawal syndrome and delirium in the PICU population, the overlap in these scoring systems presents potential difficulty in distinguishing syndromes, both clinically and for research purposes.

  8. DGCR6 at the proximal part of the DiGeorge critical region is involved in conotruncal heart defects

    Science.gov (United States)

    Gao, Wenming; Higaki, Takashi; Eguchi-Ishimae, Minenori; Iwabuki, Hidehiko; Wu, Zhouying; Yamamoto, Eiichi; Takata, Hidemi; Ohta, Masaaki; Imoto, Issei; Ishii, Eiichi; Eguchi, Mariko

    2015-01-01

    Cardiac anomaly is one of the hallmarks of DiGeorge syndrome (DGS), observed in approximately 80% of patients. It often shows a characteristic morphology, termed as conotruncal heart defects. In many cases showing only the conotruncal heart defect, deletion of 22q11.2 region cannot be detected by fluorescence in situ hybridization (FISH), which is used to detect deletion in DGS. We investigated the presence of genomic aberrations in six patients with congenital conotruncal heart defects, who show no deletion at 22q11.2 in an initial screening by FISH. In these patients, no abnormalities were identified in the coding region of the TBX1 gene, one of the key genes responsible for the phenotype of DGS. However, when copy number alteration was analyzed by high-resolution array analysis, a small deletion or duplication in the proximal end of DiGeorge critical region was detected in two patients. The affected region contains the DGCR6 and PRODH genes. DGCR6 has been reported to affect the expression of the TBX1 gene. Our results suggest that altered dosage of gene(s) other than TBX1, possibly DGCR6, may also be responsible for the development of conotruncal heart defects observed in patients with DGS and, in particular, in those with stand-alone conotruncal heart defects. PMID:27081520

  9. Child Abuse and Dissociation in Patients with Complex Regional Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Michael Williams

    1999-01-01

    Full Text Available >OBJECTIVE: In the absence of a proven medical explanation for the chronic pain syndrome Complex Regional Pain Syndrome type I (CRPS I, this study explored a hypothetical link between childhood physical and sexual abuse, and the subsequent development of CRPS I. The hypothesis predicts the existence of a subpopulation of CRPS I patients with a high frequency of dissociative experiences corresponding to a history of childhood trauma.

  10. Incidence of Complex Regional Pain Syndrome I Following Foot and Ankle Fractures Using the Budapest Criteria.

    Science.gov (United States)

    Bullen, Michael; Lang, Coran; Tran, Phong

    2016-12-01

    OBJECTIVE : Fractures are a well-recognized inciting event in the development of complex regional pain syndrome. This study aimed to prospectively determine the incidence of complex regional pain syndrome following foot and ankle fractures. METHODS : A prospective study was conducted of patients presenting to two metropolitan hospitals with plain radiograph diagnosis of fractures to the foot or ankle. Patients were initially screened by phone 3 months after injury using the validated International Association for the Study of Pain Budapest criteria. Patients who fulfilled the screening criteria were then physically examined by a pain specialist to assess clinical signs as part of the Budapest criteria. RESULTS : A total of 306 consecutive eligible patients were included. One hundred and ten patients reported at least one symptom of complex regional pain syndrome; however, only three fulfilled the minimum requirements to necessitate clinical review. Of these three, only one patient fulfilled the combination of symptom and sign criteria for a positive diagnosis according to the validated Budapest criteria. The incidence of complex regional pain syndrome following foot and ankle fracture in this study was 0.3%. CONCLUSION : Although many patients may experience vasomotor, sensory, and sudomotor disturbance following a fracture to the foot and ankle, the observed incidence of complex regional pain syndrome using a prospectively collected validated criteria is significantly lower than previously published. © 2016 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  11. An unbalanced 5;22 translocation in a patient with features of VCFS: Confirmation by FISH of loss of the DGS/VCFS critical region

    Energy Technology Data Exchange (ETDEWEB)

    Smith, J.J.; McGlothlin, J.C. [Baylor College of Medicine, Houston, TX (United States); Lindsay, E.A. [Georgia Neurological Institute, Savannah, GA (United States)

    1994-09-01

    A 14-month-old male with a history of ventricular septal defect (VSD) and cleft lip and palate (CL/P) was referred for evaluation because of growth retardation, developmental delay and hypotonia. The initial cytogenetic analysis was 45,XY,-5,-22,+der(5)t(5q:22q). Determination of breakpoints 5q35.3 and 22q11.2 were made on G-banded chromosomes with band lengths of over 550. However, with both regions being light G bands, it was difficult to tell if the break in 22 was proximal to or distal to the DiGeorge syndrome/velocardiofacial syndrome (DGS/VCFS) critical region. Since the patient had a VSD and CL/P, velocardiofacial syndrome and a deletion of the DGS/VCFS critical region was suspected. FISH analysis of the derivative chromosome was performed with a cocktail containing two probes (ONCOR), D22S75, which maps to the DGS/VCFS region in 22q11.2 and D22S39, which maps to 22q13.3 and is used as a control for the presence of chromosome 22. Three fluorescent signals were observed, two on the normal 22 and the third on the terminal end of the derivative 5 chromosome verifying the translocation of 22q to 5q. No signal was observed for D22S75 on the proximal part of the translocated segment, verifying a deletion of the DGS/VCFS region in a patient whose clinical evaluation is consistent with velocardiofacial syndrome. Experiments with additional probes are underway to determine the deletion boundaries.

  12. Cognitive-behavioral therapy for somatization and symptom syndromes: a critical review of controlled clinical trials.

    Science.gov (United States)

    Kroenke, K; Swindle, R

    2000-01-01

    Few treatments for somatization have been proven effective. In the past decade, however, clinical trials of cognitive-behavioral therapy (CBT) have been promising. Our aim was to critically review and synthesize the evidence from these trials. A search of the Medline database from 1966 through July 1999 was conducted to identify controlled trials designed to evaluate the efficacy of CBT in patients with somatization or symptom syndromes. A total of 31 controlled trials (29 randomized and 2 nonrandomized) were identified. Twenty-five studies targeted a specific syndrome (e.g. chronic fatigue, irritable bowel, pain) while 6 focused on more general somatization or hypochondriasis. Primary outcome assessment included physical symptoms, psychological distress and functional status in 28, 26 and 19 studies, respectively. Physical symptoms appeared the most responsive: CBT-treated patients improved more than control subjects in 71% of the studies and showed possibly greater improvement (i.e., a trend) in another 11% of the studies. A definite or possible advantage of CBT for reducing psychological distress was demonstrated in only 38 and 8% of studies, and for improving functional status in 47 and 26%. Group therapy and interventions as brief as 5 sessions proved efficacious. Benefits were sustained for up to 12 months. CBT can be an effective treatment for patients with somatization or symptom syndromes. Benefits can occur whether or not psychological distress is ameliorated. Since chronic symptoms are exceptionally common and most studies were conducted in referral populations, the optimal sequencing of CBT in treating primary care patients and the identification of those most likely to accept and respond to therapy should be further evaluated. Copyright 2000 S. Karger AG, Basel.

  13. Does Regional Lung Strain Correlate With Regional Inflammation in Acute Respiratory Distress Syndrome During Nonprotective Ventilation? An Experimental Porcine Study.

    Science.gov (United States)

    Retamal, Jaime; Hurtado, Daniel; Villarroel, Nicolás; Bruhn, Alejandro; Bugedo, Guillermo; Amato, Marcelo Britto Passos; Costa, Eduardo Leite Vieira; Hedenstierna, Göran; Larsson, Anders; Borges, João Batista

    2018-06-01

    It is known that ventilator-induced lung injury causes increased pulmonary inflammation. It has been suggested that one of the underlying mechanisms may be strain. The aim of this study was to investigate whether lung regional strain correlates with regional inflammation in a porcine model of acute respiratory distress syndrome. Retrospective analysis of CT images and positron emission tomography images using [F]fluoro-2-deoxy-D-glucose. University animal research laboratory. Seven piglets subjected to experimental acute respiratory distress syndrome and five ventilated controls. Acute respiratory distress syndrome was induced by repeated lung lavages, followed by 210 minutes of injurious mechanical ventilation using low positive end-expiratory pressures (mean, 4 cm H2O) and high inspiratory pressures (mean plateau pressure, 45 cm H2O). All animals were subsequently studied with CT scans acquired at end-expiration and end-inspiration, to obtain maps of volumetric strain (inspiratory volume - expiratory volume)/expiratory volume, and dynamic positron emission tomography imaging. Strain maps and positron emission tomography images were divided into 10 isogravitational horizontal regions-of-interest, from which spatial correlation was calculated for each animal. The acute respiratory distress syndrome model resulted in a decrease in respiratory system compliance (20.3 ± 3.4 to 14.0 ± 4.9 mL/cm H2O; p < 0.05) and oxygenation (PaO2/FIO2, 489 ± 80 to 92 ± 59; p < 0.05), whereas the control animals did not exhibit changes. In the acute respiratory distress syndrome group, strain maps showed a heterogeneous distribution with a greater concentration in the intermediate gravitational regions, which was similar to the distribution of [F]fluoro-2-deoxy-D-glucose uptake observed in the positron emission tomography images, resulting in a positive spatial correlation between both variables (median R = 0.71 [0.02-0.84]; p < 0.05 in five of seven animals

  14. Effects of white-nose syndrome on regional population patterns of 3 hibernating bat species.

    Science.gov (United States)

    Ingersoll, Thomas E; Sewall, Brent J; Amelon, Sybill K

    2016-10-01

    Hibernating bats have undergone severe recent declines across the eastern United States, but the cause of these regional-scale declines has not been systematically evaluated. We assessed the influence of white-nose syndrome (an emerging bat disease caused by the fungus Pseudogymnoascus destructans, formerly Geomyces destructans) on large-scale, long-term population patterns in the little brown myotis (Myotis lucifugus), the northern myotis (Myotis septentrionalis), and the tricolored bat (Perimyotis subflavus). We modeled population trajectories for each species on the basis of an extensive data set of winter hibernacula counts of more than 1 million individual bats from a 4-state region over 13 years and with data on locations of hibernacula and first detections of white-nose syndrome at each hibernaculum. We used generalized additive mixed models to determine population change relative to expectations, that is, how population trajectories differed with a colony's infection status, how trajectories differed with distance from the point of introduction of white-nose syndrome, and whether declines were concordant with first local observation of the disease. Population trajectories in all species met at least one of the 3 expectations, but none met all 3. Our results suggest, therefore, that white-nose syndrome has affected regional populations differently than was previously understood and has not been the sole cause of declines. Specifically, our results suggest that in some areas and species, threats other than white-nose syndrome are also contributing to population declines, declines linked to white-nose syndrome have spread across large geographic areas with unexpected speed, and the disease or other threats led to declines in bat populations for years prior to disease detection. Effective conservation will require further research to mitigate impacts of white-nose syndrome, renewed attention to other threats to bats, and improved surveillance efforts to ensure

  15. Scaled parametric equation of state for steam in the critical region

    International Nuclear Information System (INIS)

    Murphy, T.A.; Sengers, J.V.

    1975-01-01

    The anomalous thermodynamic behavior of fluids near the critical point can be described in terms of scaling laws. In recent years a parametric equation of state, the so-called Linear Model, has been proposed that satisfies the scaling laws and contains only a small number of adjustable parameters. It is shown that the Linear Model yields a satisfactory representation of the experimental P-V-T data for steam in the critical region. (29 references)

  16. The U(1)-Higgs model: critical behaviour in the confining-Higgs region

    International Nuclear Information System (INIS)

    Alonso, J.L.; Azcoiti, V.; Campos, I.; Ciria, J.C.; Cruz, A.; Iniguez, D.; Lesmes, F.; Piedrafita, C.; Rivero, A.; Tarancon, A.; Badoni, D.; Fernandez, L.A.; Munoz Sudupe, A.; Ruiz-Lorenzo, J.J.; Gonzalez-Arroyo, A.; Martinez, P.; Pech, J.; Tellez, P.

    1993-01-01

    We study numerically the critical properties of the U(1)-Higgs lattice model, with fixed Higgs modulus, in the region of small gauge coupling where the Higgs and confining phases merge. We find evidence for a first-order transition line that ends in a second-order point. By means of a rotation in parameter space we introduce thermodynamic magnitudes and critical exponents in close resemblance with simple models that show analogous critical behaviour. The measured data allow us to fit the critical exponents finding values in agreement with the mean-field prediction. The location of the critical point and the slope of the first-order line are accurately measured. (orig.)

  17. Identity and representation in Russia's regions: Adopting a critical theory perspective

    Directory of Open Access Journals (Sweden)

    Andrey Makarychev

    2012-07-01

    Full Text Available In this paper, the author seeks to find pathways of extrapolating the critical potential of post-structuralist reasoning to the study of Russia's domestic regions' policies. He argues that ideas, norms and rhetorical frames are important ideational arguments to explain policy outcomes in specific Russia's region and in the whole system of Russian federalism. Analysis of Russian regionalism, therefore, can be enriched by engaging with and adopting the new concepts and tools bringing attention to the power of regional identities as exemplified by different types of discourses.

  18. Investigation of regional cerebral blood flow in alcoholic Korsakoff's syndrome with 123I-IMP SPECT

    International Nuclear Information System (INIS)

    Nakamura, Makoto; Nomura, Soichiro; Kato, Motoichiro; Nakazawa, Tsuneyuki.

    1995-01-01

    Regional cerebral blood flow (rCBF) was determined quantitatively by single photon emission computed tomography (SPECT) using N-isopropyl-p-[ 123 I]iodoamphetamine ( 123 I-IMP) in 6 patients with alcoholic Korsakoff's syndrome (A group). The findings were compared with concurrently available findings from 6 alcoholic patients with no evidence of cerebral disordres such as Korsakoff's syndrome and dementia (B group) and 4 healthy persons (C group). In both A and B groups, diffuse decrease in blood flow was significantly observed in the entire brain, as compared with the C group; no significant difference in the decreased blood flow existed between the A and B groups. According to the WAIS results, the patients in the A group were classified as 'typical Korsakoff's syndrome' (full IQ of 90 or more) and as 'serious Korsakoff's syndrome' (full IQ of 89 or less). rCBF in the thalamus was significantly lower in the A group of patients with typical Korsakoff's syndrome than the B group. These findings may reflect the variety of alcoholic Korsakoff's syndrome. This may also have an implication for the possible classification of several types in this syndrome. (N.K.)

  19. [Coffin-Siris syndrome. Critical study of the literature apropos of a case].

    Science.gov (United States)

    Foasso, M F; Hermier, M; Descos, B; Collet, J P; Perron, F

    1983-03-01

    The authors report a case of the Coffin Siris syndrome which associates a ungueo-digital syndrome (special by the bilateral aplasia or severe hypoplasia of nails and third phalanx of fifth toes and fingers) to other anomalies: facies with thinly fine hairs contrasting with bushy and dense eyebrows and body hypertrichosis, hypotonia and mental retardation. The connections of the Coffin Siris syndrome with the trisomy 9 p+ syndrome and the fetal hydantoïn syndrome are discussed.

  20. Persistent Delirium in Chronic Critical Illness as a Prodrome Syndrome before Death.

    Science.gov (United States)

    DeForest, Anna; Blinderman, Craig D

    2017-05-01

    Chronic critical illness (CCI) patients have poor functional outcomes, high risk of mortality, and significant sequelae, including delirium and cognitive dysfunction. The prognostic significance of persistent delirium in patients with CCI has not been well described. We report a case of a patient with CCI following major cardiac surgery who was hemodynamically stable following a long course in the cardiothoracic intensive care unit (CTICU), but had persistent and unremitting delirium. Despite both pharmacological and nonpharmacological approaches to improve his delirium, the patient ultimately continued to have symptoms of delirium and subsequently died in the CTICU. Efforts to reconsider the goals of care, given his family's understanding of his values, were met with resistance as his cardiothoracic surgeon believed that he had a reasonable chance of recovery since his organs were not in failure. This case description raises the question of whether we should consider persistent delirium as a prodrome syndrome before death in patients with CCI. Study and analysis of a case of a patient with CCI following major cardiothoracic surgery who was hemodynamically stable with persistent delirium. Further studies of the prevalence and outcomes of prolonged or persistent agitated delirium in patients with chronic critical illness are needed to provide prognostic information that can assist patients and families in receiving care that accords with their goals and values.

  1. Calculation of critical fault recovery time for nonlinear systems based on region of attraction analysis

    DEFF Research Database (Denmark)

    Tabatabaeipour, Mojtaba; Blanke, Mogens

    2014-01-01

    of a system. It must be guaranteed that the trajectory of a system subject to fault remains in the region of attraction (ROA) of the post-fault system during this time. This paper proposes a new algorithm to compute the critical fault recovery time for nonlinear systems with polynomial vector elds using sum...

  2. Universities and Regional Development: A Critical Assessment of Tensions and Contradictions. International Studies in Higher Education

    Science.gov (United States)

    Pinheiro, Romulo, Ed.; Benneworth, Paul, Ed.; Jones, Glen A., Ed.

    2012-01-01

    Universities are under increasing pressure to help promote socio-economic growth in their local communities. However until now, no systematic, critical attention has been paid to the factors and mechanisms that currently make this process so daunting. In Universities and Regional Development, scholars from Europe, the Americas, Africa, and Asia…

  3. Age is a critical risk factor for severe fever with thrombocytopenia syndrome.

    Directory of Open Access Journals (Sweden)

    Shujun Ding

    Full Text Available Severe Fever with Thrombocytopenia Syndrome (SFTS is an emerging infectious disease in East Asia. SFTS is a tick borne hemorrhagic fever caused by SFTSV, a new bunyavirus named after the syndrome. We investigated the epidemiology of SFTS in Laizhou County, Shandong Province, China.We collected serum specimens of all patients who were clinically diagnosed as suspected SFTS cases in 2010 and 2011 in Laizhou County. The patients' serum specimens were tested for SFTSV by real time fluorescence quantitative PCR (RT-qPCR. We collected 1,060 serum specimens from healthy human volunteers by random sampling in Laizhou County in 2011. Healthy persons' serum specimens were tested for specific SFTSV IgG antibody by ELISA.71 SFTS cases were diagnosed in Laizhou County in 2010 and 2011, which resulted in the incidence rate of 4.1/100,000 annually. The patients ranged from 15 years old to 87 years old and the median age of the patients were 59 years old. The incidence rate of SFTS was significantly higher in patients over 40 years old and fatal cases only occurred in patients over 50 years old. 3.3% (35/1,060 of healthy people were positive to SFTSV IgG antibody. The SFTSV antibody positive rate was not significantly different among people at different age groups.Our results revealed that seroprevalence of SFTSV in healthy people in Laizhou County was not significantly different among age groups, but SFTS patients were mainly elderly people, suggesting that age is the critical risk factor or determinant for SFTS morbidity and mortality.

  4. Samara region experience in organization of emergency care system for patients with acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Duplyakov D.V.

    2015-09-01

    Full Text Available The article presents the general features of healthcare program for patients with acute coronary syndrome which started in Samara region in 2007. Some results and perspectives of future development of Samara “myocardial infarction network” are elucidated.

  5. Complex regional pain syndrome related movement disorders : studies on pathophysiology and therapy.

    NARCIS (Netherlands)

    Munts, Alexander Gerard

    2011-01-01

    Complex regional pain syndrome (CRPS) may occur after trauma, usually to one limb, and is characterised by pain and disturbed blood flow, temperature regulation and motor control. Knowledge on CRPS and its movement disorders is scarce. Dysfunction in small nerve fiber processing was found in CRPS

  6. Mitochondrial dysfunction in muscle tissue of complex regional pain syndrome type I patients

    NARCIS (Netherlands)

    Tan, E.C.T.H.; Janssen, A.J.W.M.; Roestenberg, P.M.H.; Heuvel, L.P.W.J. van den; Goris, R.J.A.; Rodenburg, R.J.T.

    2011-01-01

    Reactive oxygen species (ROS) are known to be involved in the pathophysiology of complex regional pain syndrome type I (CRPS I). Since the mitochondrial respiratory chain is a major source of ROS, we hypothesized that mitochondria play a role in the pathophysiology of CRPS I. The hypothesis was

  7. Mannitol as salvage treatment for Complex Regional Pain Syndrome Type I.

    NARCIS (Netherlands)

    Tan, E.C.T.H.; Tacken, M.C.; Groenewoud, J.M.M.; Goor, H. van; Frolke, J.P.M.

    2010-01-01

    INTRODUCTION: Complex Regional Pain Syndrome Type I (CRPS I) is a continuation of symptoms and signs due to a pathological exaggerated reaction in an extremity of the human body after an injury or operation. Although the clinical picture of CRPS I in the majority of patients is well known, the

  8. Evidence for local inflammation in complex regional pain syndrome type 1

    Directory of Open Access Journals (Sweden)

    Frank J. P. M. Huygen

    2002-01-01

    Full Text Available Background: The pathophysiology of complex regional pain syndrome type 1 (CRPS 1 is still a matter of debate. Peripheral afferent, efferent and central mechanisms are supposed. Based on clinical signs and symptoms (e.g. oedema, local temperature changes and chronic pain local inflammation is suspected.

  9. Dystonia in complex regional pain syndrome : clinical, pathophysiological and therapeutic aspects

    NARCIS (Netherlands)

    Rijn, Monica Adriana van

    2010-01-01

    The clinical characteristics of Complex Regional Pain Syndrome (CRPS) are defined by pain and various combinations of sensory disturbances, autonomic features, and sudomotor and trophic changes. Furthermore, patients with CRPS may suffer from movement disorders, of which dystonia is the most

  10. Muscle Hyperalgesia Correlates With Motor Function in Complex Regional Pain Syndrome Type 1

    NARCIS (Netherlands)

    van Rooijen, Diana E.; Marinus, Johan; Schouten, Alfred Christiaan; Noldus, Lucas P.J.J.; van Hilten, Jacobus J.

    2013-01-01

    At present it is unclear if disturbed sensory processing plays a role in the development of the commonly observed motor impairments in patients with complex regional pain syndrome (CRPS). This study aims to investigate the relation between sensory and motor functioning in CRPS patients with and

  11. Genetic HLA Associations in Complex Regional Pain Syndrome With and Without Dystonia

    NARCIS (Netherlands)

    van Rooijen, D.E.; Roelen, D.L.; Verduijn, W.; Haasnoot, G.W.; Huygen, F.J.P.M.; Perez, R.S.G.M.; Claas, F.H.J.; Marinus, J.; van Hilten, J.J.; van den Maagdenberg, A.M.J.M.

    2012-01-01

    We previously showed evidence for a genetic association of the human leukocyte antigen (HLA) system and complex regional pain syndrome (CRPS) with dystonia. Involvement of the HLA system suggests that CRPS has a genetic component with perturbed regulation of inflammation and neuroplasticity as

  12. Informed Decision-Making Regarding Amputation for Complex Regional Pain Syndrome Type I

    NARCIS (Netherlands)

    Bodde, Marlies I.; Dijkstra, Pieter U.; Schrier, Michiel; van den Dungen, Johannes; den Dunnen, Wilfred E.; Geertzen, Joannes

    2014-01-01

    Background: Literature on complex regional pain syndrome type I (CRPS-I) discussing the decision to amputate or not, the level of amputation, or the timing of the amputation is scarce: We evaluated informed decision-making regarding amputation for CRPS-I. Methods: We describe our findings in a

  13. Rehabilitation of a female dancer with patellofemoral pain syndrome: applying concepts of regional interdependence in practice.

    Science.gov (United States)

    Welsh, Caitlyn; Hanney, William J; Podschun, Laura; Kolber, Morey J

    2010-06-01

    Due to complex movements and high physical demands, dance is often associated with a multitude of impairments including pain of the low back, pelvis, leg, knee, and foot. This case report provides an exercise progression, emphasizing enhancement of strength and neuromuscular performance using the concept of regional interdependence in a 17 year old female dancer with patellofemoral pain syndrome.

  14. Cerebral activation during motor imagery in complex regional pain syndrome type 1 with dystonia

    NARCIS (Netherlands)

    Gieteling, Esther W.; van Rijn, Monique A.; de Jong, Bauke M.; Hoogduin, Johannes M.; Renken, Remco; van Hilten, Jacobus J.; Leenders, Klaus L.

    The pathogenesis of dystonia in Complex Regional Pain Syndrome type 1 (CRPS-1) is unclear. In primary dystonia, functional magnetic resonance imaging (fMRI) has revealed changes in cerebral networks during execution of movement. The aim of this study was to determine cerebral network function in

  15. Effects of white-nose syndrome on regional population patterns of 3 hibernating bat species

    Science.gov (United States)

    Thomas E. Ingersoll; Brent J. Sewall; Sybill K. Amelon

    2016-01-01

    Hibernating bats have undergone severe recent declines across the eastern United States, but the cause of these regional-scale declines has not been systematically evaluated. We assessed the influence of white-nose syndrome (an emerging bat disease caused by the fungus Pseudogymnoascus destructans, formerly Geomyces destructans...

  16. Stressful life events and psychological dysfunction in complex regional pain syndrome type I

    NARCIS (Netherlands)

    Geertzen, JHB; de Bruijn-Kofman, AT; de Bruijn, HP; van de Wiel, HBM; Dijkstra, PU

    Objective: To determine to what extent stressful life events and psychological dysfunction play a role in the pathogenesis of Complex Regional Pain Syndrome type I (CRPS). Design: A comparative study between a CRPS group and a control group. Stressful life events and psychological dysfunction

  17. Resilience in patients with amputation because of Complex Regional Pain Syndrome type I

    NARCIS (Netherlands)

    Bodde, Marlies I.; Schrier, Ernst; Krans, Hilde K.; Geertzen, J.H.B.; Dijkstra, Pieter U.

    2014-01-01

    Purpose: Although controversial, an amputation for longstanding and therapy-resistant Complex Regional Pain Syndrome Type I (CRPS-I) may improve quality of life and pain intensity. Resilience, the way people deal with adversity in a positive way may be related to these positive outcomes. This study

  18. Optokinetic stimulation increases limb pain and forehead hyperalgesia in complex regional pain syndrome

    DEFF Research Database (Denmark)

    Knudsen, Lone F.; Drummond, Peter D.

    2015-01-01

    BACKGROUND: Ambiguous visual stimuli increase limb pain in patients with complex regional pain syndrome (CRPS), possibly due to afferent sensory feedback conflicts. Conflicting sensory stimuli can also generate unpleasant sensations in healthy people such as during motion sickness. We wanted to i...

  19. Regionalization in the SUS: implementation process, challenges and perspectives in the critical view of system managers.

    Science.gov (United States)

    Carvalho, Andre Luis Bonifácio de; Jesus, Washington Luiz Abreu de; Senra, Isabel Maria Vilas Boas

    2017-04-01

    This article examines the regionalization process in the Brazilian Health System, identifying frameworks and challenges of this process from critical dialogue on the subject, contextualized by the experience of the management system and in the light of an established theoretical debate in the last decade. We used the thematic content analysis of legal and documentary surveys of the regionalization process in SUS, collated by elements of the historical and political context in the period. As evidence, it appears that the regionalization process has been incremental decentralization/deconcentration of management and health actions and services. There are important challenges, particularly in relation to ensuring access and system governance structure, which contributes to critical thinking and construction of new perspectives by those who lead their implementation.

  20. Human papillomavirus vaccines, complex regional pain syndrome, postural orthostatic tachycardia syndrome, and autonomic dysfunction - a review of the regulatory evidence from the European Medicines Agency

    DEFF Research Database (Denmark)

    Jefferson, Tom; Jørgensen, Lars

    2017-01-01

    Recent concerns about a possible association between exposure of young women to human papillomavirus (HPV) vaccines and two "dysautonomic syndromes" (a collection of signs and symptoms thought to be caused by autoimmunity) - complex regional pain syndrome (CRPS) and postural orthostatic tachycardia...

  1. Inversion of the Williams syndrome region is a common polymorphism found more frequently in parents of children with Williams syndrome.

    Science.gov (United States)

    Hobart, Holly H; Morris, Colleen A; Mervis, Carolyn B; Pani, Ariel M; Kistler, Doris J; Rios, Cecilia M; Kimberley, Kendra W; Gregg, Ronald G; Bray-Ward, Patricia

    2010-05-15

    Williams syndrome (WS) is a multisystem disorder caused by deletion of about 1.55 Mb of DNA (including 26 genes) on chromosome 7q11.23, a region predisposed to recombination due to its genomic structure. Deletion of the Williams syndrome chromosome region (WSCR) occurs sporadically. To better define chance for familial recurrence and to investigate the prevalence of genomic rearrangements of the region, 257 children with WS and their parents were studied. We determined deletion size in probands by metaphase FISH, parent-of-origin of the deleted chromosome by molecular genetic methods, and inversion status of the WSCR in both parents by interphase FISH. The frequency of WSCR inversion in the transmitting parent group was 24.9%. In contrast, the rate of inversion in the non-transmitting parent group (a reasonable estimate of the rate in the general population) was 5.8%. There were no significant gender differences with respect to parent-of-origin for the deleted chromosome or the incidence of the inversion polymorphism. There was no difference in the rate of spontaneous abortion for mothers heterozygous for the WSCR inversion relative to mothers without the inversion. We calculate that for a parent heterozygous for a WSCR inversion, the chance to have a child with WS is about 1 in 1,750, in contrast to the 1 in 9,500 chance for a parent without an inversion.

  2. Complex regional pain syndrome type 1 mimicking Raynaud’s phenomenon

    Directory of Open Access Journals (Sweden)

    Serpil Tuna

    2014-09-01

    Full Text Available Complex regional pain syndrome type 1 (CRPS-1 is a chronic pain syndrome characterized by severe pain, swelling, autonomic dysfunction and dystrophic changes in affected extremity. RSDS is a rare disease in children and usually occurs after trauma, however, without trauma may also occur. We were detected CRPS-1 activated by cold and stress and characterized by recurrent attacks in the bilateral upper extremities in 14 year-old girl, which is similar to Raynaud’s phenomenon. We present this case with the literature because of its rarity and atypical course.

  3. Treatment of Complex Regional Pain Syndrome (CRPS) using low dose naltrexone (LDN).

    Science.gov (United States)

    Chopra, Pradeep; Cooper, Mark S

    2013-06-01

    Complex Regional Pain Syndrome (CRPS) is a neuropathic pain syndrome, which involves glial activation and central sensitization in the central nervous system. Here, we describe positive outcomes of two CRPS patients, after they were treated with low-dose naltrexone (a glial attenuator), in combination with other CRPS therapies. Prominent CRPS symptoms remitted in these two patients, including dystonic spasms and fixed dystonia (respectively), following treatment with low-dose naltrexone (LDN). LDN, which is known to antagonize the Toll-like Receptor 4 pathway and attenuate activated microglia, was utilized in these patients after conventional CRPS pharmacotherapy failed to suppress their recalcitrant CRPS symptoms.

  4. Post-traumatic complex regional pain syndrome: clinical features and epidemiology

    Science.gov (United States)

    Ratti, Chiara; Nordio, Andrea; Resmini, Giuseppina; Murena, Luigi

    2015-01-01

    Summary Complex Regional Pain Syndrome (CRPS) is a chronic pain condition that occurs after a tissue injury (fractures, sprain, surgery) of the upper or lower extremities. A clear pathophysiological mechanism has not been established yet and different patterns are considered to play a role in the genesis of the disease. The diagnosis is made by different diagnosis criteria and a gold standard has not been established yet. Incidence of CRPS is unclear and large prospective studies on the incidence and prevalence of CRPS are scarce. The aim of this review is to give an overview on the prevalent data regarding this chronic syndrome. PMID:27134626

  5. Pulsed Radiofrequency Treatment of Complex Regional Pain Syndrome: A Case Series

    Directory of Open Access Journals (Sweden)

    Vlad Djuric

    2014-01-01

    Full Text Available BACKGROUND: Various forms of sympathetic chain neurolysis (sympathectomy have, at one time or another, held promise as effective treatment options for complex regional pain syndrome (CRPS. Complications, such as worsening pain and the development of new pain syndromes, have prevented sympathectomy from emerging as a standard intervention. In an effort to avoid poor outcomes associated with neurolysis, pulsed radiofrequency (PRF has been proposed as a potential treatment alternative for a number of chronic neuropathic pain states, including some forms of CRPS.

  6. [Hantavirus pulmonary syndrome (Rio Mamore virus) in the Peruvian Amazon region].

    Science.gov (United States)

    Casapía, Martín; Mamani, Enrique; García, María P; Miraval, María L; Valencia, Pedro; Quino, Alberto H; Alvarez, Carlos; Donaires, Luis F

    2012-01-01

    Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analysis of a viral genome fragment and a histopathological evaluation were conducted. Both patients developed adult respiratory distress syndrome and refractory shock. A patient died and another one recovered 12 days later.

  7. Neonatal and pediatric regionalized systems in pediatric emergency mass critical care.

    Science.gov (United States)

    Barfield, Wanda D; Krug, Steven E; Kanter, Robert K; Gausche-Hill, Marianne; Brantley, Mary D; Chung, Sarita; Kissoon, Niranjan

    2011-11-01

    Improved health outcomes are associated with neonatal and pediatric critical care in well-organized, cohesive, regionalized systems that are prepared to support and rehabilitate critically ill victims of a mass casualty event. However, present systems lack adequate surge capacity for neonatal and pediatric mass critical care. In this document, we outline the present reality and suggest alternative approaches. In May 2008, the Task Force for Mass Critical Care published guidance on provision of mass critical care to adults. Acknowledging that the critical care needs of children during disasters were unaddressed by this effort, a 17-member Steering Committee, assembled by the Oak Ridge Institute for Science and Education with guidance from members of the American Academy of Pediatrics, convened in April 2009 to determine priority topic areas for pediatric emergency mass critical care recommendations.Steering Committee members established subcommittees by topic area and performed literature reviews of MEDLINE and Ovid databases. The Steering Committee produced draft outlines through consensus-based study of the literature and convened October 6-7, 2009, in New York, NY, to review and revise each outline. Eight draft documents were subsequently developed from the revised outlines as well as through searches of MEDLINE updated through March 2010.The Pediatric Emergency Mass Critical Care Task Force, composed of 36 experts from diverse public health, medical, and disaster response fields, convened in Atlanta, GA, on March 29-30, 2010. Feedback on each manuscript was compiled and the Steering Committee revised each document to reflect expert input in addition to the most current medical literature. States and regions (facilitated by federal partners) should review current emergency operations and devise appropriate plans to address the population-based needs of infants and children in large-scale disasters. Action at the state, regional, and federal levels should address

  8. A statistical study of current-sheet formation above solar active regions based on selforganized criticality

    Science.gov (United States)

    Dimitropoulou, M.; Isliker, H.; Vlahos, L.; Georgoulis, M.; Anastasiadis, A.; Toutountzi, A.

    2013-09-01

    We treat flaring solar active regions as physical systems having reached the self-organized critical state. Their evolving magnetic configurations in the low corona may satisfy an instability criterion, related to the excession of a specific threshold in the curl of the magnetic field. This imposed instability criterion implies an almost zero resistivity everywhere in the solar corona, except in regions where magnetic-field discontinuities and. hence, local currents, reach the critical value. In these areas, current-driven instabilities enhance the resistivity by many orders of magnitude forming structures which efficiently accelerate charged particles. Simulating the formation of such structures (thought of as current sheets) via a refined SOC cellular-automaton model provides interesting information regarding their statistical properties. It is shown that the current density in such unstable regions follows power-law scaling. Furthermore, the size distribution of the produced current sheets is best fitted by power laws, whereas their formation probability is investigated against the photospheric magnetic configuration (e.g. Polarity Inversion Lines, Plage). The average fractal dimension of the produced current sheets is deduced depending on the selected critical threshold. The above-mentioned statistical description of intermittent electric field structures can be used by collisional relativistic test particle simulations, aiming to interpret particle acceleration in flaring active regions and in strongly turbulent media in astrophysical plasmas. The above work is supported by the Hellenic National Space Weather Research Network (HNSWRN) via the THALIS Programme.

  9. Light Scattering Tests of Fundamental Theories of Transport Properties in the Critical Region

    Science.gov (United States)

    Gammon, R. W.; Moldover, M. R.

    1985-01-01

    The objective of this program is to measure the decay rates of critical density fluctuations in a simple fluid (xenon) very near its liquid-vapor critical point using laser light scattering and photon correlation spectroscopy. Such experiments have been severely limited on Earth by the presence of gravity which causes large density gradients in the sample when the compressibility diverges approaching the critical point. The goal is to measure decay rates deep in the critical region where the scaled wavevector is the order of 1000. This will require loading the sample to 0.01% of the critical density and taking data as close as 3 microKelvin to the critical temperature (Tc = 289.72 K). Other technical problems have to be addressed such as multiple scattering and the effect of wetting layers. The ability to avoid multiple scattering by using a thin sample (100 microns) was demonstrated, as well as a temperature history which can avoid wetting layers satisfactory temperature control and measurement, and accurate sample loading. Thus the questions of experimental art are solved leaving the important engineering tasks of mounting the experiment to maintain alignment during flight and automating the state-of-the-art temperature bridges for microcomputer control of the experiment.

  10. An Official Critical Care Societies Collaborative Statement-Burnout Syndrome in Critical Care Health-care Professionals: A Call for Action.

    Science.gov (United States)

    Moss, Marc; Good, Vicki S; Gozal, David; Kleinpell, Ruth; Sessler, Curtis N

    2016-07-01

    Burnout syndrome (BOS) occurs in all types of health-care professionals and is especially common in individuals who care for critically ill patients. The development of BOS is related to an imbalance of personal characteristics of the employee and work-related issues or other organizational factors. BOS is associated with many deleterious consequences, including increased rates of job turnover, reduced patient satisfaction, and decreased quality of care. BOS also directly affects the mental health and physical well-being of the many critical care physicians, nurses, and other health-care professionals who practice worldwide. Until recently, BOS and other psychological disorders in critical care health-care professionals remained relatively unrecognized. To raise awareness of BOS, the Critical Care Societies Collaborative (CCSC) developed this call to action. The present article reviews the diagnostic criteria, prevalence, causative factors, and consequences of BOS. It also discusses potential interventions that may be used to prevent and treat BOS. Finally, we urge multiple stakeholders to help mitigate the development of BOS in critical care health-care professionals and diminish the harmful consequences of BOS, both for critical care health-care professionals and for patients. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  11. Improving mortality outcomes of Stevens Johnson syndrome/toxic epidermal necrolysis: A regional burns centre experience.

    Science.gov (United States)

    Nizamoglu, M; Ward, J A; Frew, Q; Gerrish, H; Martin, N; Shaw, A; Barnes, D; Shelly, O; Philp, B; El-Muttardi, N; Dziewulski, P

    2018-05-01

    Stevens Johnson Syndrome/toxic epidermal necrolysis (SJS/TEN) are rare, potentially fatal desquamative disorders characterised by large areas of partial thickness skin and mucosal loss. The degree of epidermal detachment that occurs has led to SJS/TEN being described as a burn-like condition. These patients benefit from judicious critical care, early debridement and meticulous wound care. This is best undertaken within a multidisciplinary setting led by clinicians experienced in the management of massive skin loss and its sequelae. In this study, we examined the clinical outcomes of SJS/TEN overlap & TEN patients managed by our regional burns service over a 12-year period. We present our treatment model for other burn centres treating SJS/TEN patients. A retrospective case review was performed for all patients with a clinical diagnosis of TEN or SJS/TEN overlap admitted to our paediatric and adult burns centre between June 2004 and December 2016. Patient demographics, percentage total body surface area (%TBSA), mucosal involvement, causation, severity of illness score (SCORTEN), length of stay and survival were appraised with appropriate statistical analysis performed using Graph Pad Prism 7.02 Software. During the study period, 42 patients (M26; F: 16) with TEN (n=32) and SJS/TEN overlap (n=10) were managed within our burns service. Mean %TBSA of cutaneous involvement was 57% (range 10-100%) and mean length of stay (LOS) was 27 days (range 1-144 days). We observed 4 deaths in our series compared to 16 predicted by SCORTEN giving a standardised mortality ratio (SMR) of 24%. Management in our burns service with an aggressive wound care protocol involving debridement of blistered epidermis and wound closure with synthetic and biological dressings seems to have produced benefits in mortality when compared to predicted outcomes. Copyright © 2017 Elsevier Ltd and ISBI. All rights reserved.

  12. Is physiotherapy effective for children with complex regional pain syndrome type 1?

    Science.gov (United States)

    Bialocerkowski, Andrea E; Daly, Anne

    2012-01-01

    This study synthesized current research evidence on the effectiveness of physiotherapy for the management of children diagnosed with complex regional pain syndrome type 1 (CRPS-1), to provide up-to-date physiotherapy treatment recommendations, and to identify areas that require further investigation. Nine electronic databases were searched for quantitative studies that evaluated the effect of physiotherapy on children with CRPS-1. The methodological quality of the studies was evaluated using the Critical Review Form. Data were extracted regarding the study design, participant characteristics, types of outcome measures used, and physiotherapy technique used and its effectiveness. The search strategy identified 303 potential studies, of which 12 met the inclusion criteria (1 randomized controlled trial, 1 comparative study, 10 case series). The methodological quality of all studies was rated as poor to fair. The "stand-alone" value of physiotherapy could not be determined as physiotherapy was prescribed in conjunction with psychological and medical interventions. There is low volume and poor-to-fair quality evidence which suggests that physiotherapy prescribed with other interventions may lead to short-term improvement in the signs and symptoms of CRPS-1 or functional ability in children with CRPS-1, and the relapse rate may be moderately high. High-quality studies are required in this area. These studies should evaluate a package of care (which includes physiotherapy); they should investigate the effects of physiotherapy treatments that have proven effectiveness in adults with CRPS-1; they should use psychometrically sound measures to evaluate outcome; and the nature of physiotherapy should be detailed in future publications to enable replication in the clinical setting.

  13. TRITON, 3-D Multi-Region Neutron Diffusion Burnup with Criticality Search

    International Nuclear Information System (INIS)

    1974-01-01

    1 - Nature of physical problem solved: TRITON is a multigroup diffusion depletion program in three dimensions (x,y,z). In addition to the straight K eff calculation, three types of criticality searches are possible - diluted control isotope search, region-wise smeared control isotope search, region-wise smeared control isotope search, region-wise smeared control isotope boundary search (the control isotope can be smeared over one region or over a group of regions called a control bank). The depletion equations are solved region-wise. More than one microscopic cross section library can be used in the various regions of the reactor. The same is true for self-shielding factors. Such sets of data can be changed at pre-determined time steps. 2 - Method of solution: The mathematical model employed for the solution of the finite difference equations, which is derived from a seven-point approximation of diffusion equations, is an on-line Chebyshev semi- iterative method. 3 - Restrictions on the complexity of the problem: Maximum number of: library sets: 1; self-shielding sets: 10; compositions: 100; self-shielding coefficients: 6000; groups: 10; fuel isotopes: 30; fission products: 29; isotopes: 50; burnable isotopes: 40; control banks: 100; mesh points: 15000; regions: 400; time steps: 100; control areas: 100; small time steps: 200; elements in the control list: 400; x planes: 100; y planes: 100; z planes: 100

  14. Neuropeptides, neurogenic inflammation and complex regional pain syndrome (CRPS).

    Science.gov (United States)

    Birklein, Frank; Schmelz, Martin

    2008-06-06

    This review explains symptoms and nature of neuropeptide signaling and its importance for clinical symptoms of CRPS. Neurogenic inflammation regularly accompanies excitation of primary afferent nociceptors. It has two major components-plasma extravasation and vasodilatation. The most important mediators are the calcitonin gene-related peptide (CGRP) and substance P (SP). After peripheral trauma immune reaction (e.g. cytokines) and the attempts of the tissue to regenerate (e.g. growth factors) sensitize nociceptors and amplify neurogenic inflammation. This cascade of events has been demonstrated in rat models of CRPS. Clinical findings in these animals strongly resemble clinical findings in CRPS, and can be prevented by anti-cytokine and anti-neuropeptide treatment. In CRPS patients, there is meanwhile also plenty of evidence that neurogenic inflammation contributes to clinical presentation. Increased cytokine production was demonstrated, as well as facilitated neurogenic inflammation. Very recently even "non-inflammatory" signs of CRPS (hyperhidrosis, cold skin) have been linked to neuropeptide signaling. Surprisingly, there was even moderately increased neurogenic inflammation in unaffected body regions. This favors the possibility that CRPS patients share genetic similarities. The future search for genetic commonalities will help us to further unravel the "mystery" CRPS.

  15. Intersecting Itineraries Beyond the Strada Novissima: The Converging Authorship of Critical Regionalism

    Directory of Open Access Journals (Sweden)

    Stylianos Giamarelos

    2016-07-01

    Full Text Available While the 1980 Venice Biennale is usually understood as the exhibition that crystallised postmodernism as a style of historicist eclecticism, the event also acted as a catalyst for the eventual convergence of alternative architectural sensibilities and ideas. This article shows how critical regionalism emerged when the physical and intellectual trajectories of British historian Kenneth Frampton and the Greek architects Suzana Antonakaki and Dimitris Antonakakis intersected in the aftermath of the Biennale. Offering an alternative way out of the contemporaneous crisis of modernism, this open-ended and extrovert regionalism that opposed static cultural insularities is thus the discursive footprint of architectural sensibilities travelling through cultures.

  16. Psychiatric and neuropsychological issues in Marfan syndrome: A critical review of the literature.

    Science.gov (United States)

    Gritti, Antonella; Pisano, Simone; Catone, Gennaro; Iuliano, Raffaella; Salvati, Tiziana; Gritti, Paolo

    2015-01-01

    The cooccurrence of Marfan syndrome and psychiatric disorders has been reported for many years. Furthermore, neuropsychological deficits have been shown to be associated with Marfan syndrome. The aim of the present article is to summarize findings from the sparse studies and case reports available. The results hold clinical and therapeutic implications and suggest that psychological and neuropsychological domains in Marfan syndrome patients should be carefully assessed. In particular, some patients may require specific rehabilitation programs. On this basis, a multidisciplinary approach to Marfan syndrome treatment seems mandatory. © The Author(s) 2015.

  17. Rule base system for identification of patients with specific critical care syndromes: The "sniffer" for acute lung injury.

    Science.gov (United States)

    Herasevich, V; Yilmaz, M; Khan, H; Chute, C G; Gajic, O

    2007-10-11

    Early detection of specific critical care syndromes, such as sepsis or acute lung injury (ALI)is essential for timely implementation of evidence based therapies. Using a near-real time copy of the electronic medical records ("ICU data mart") we developed and validated custom electronic alert (ALI"sniffer") in a cohort of 485 critically ill medical patients. Compared with the gold standard of prospective screening, ALI "sniffer" demonstrated good sensitivity, 93% (95% CI 90 to 95) and specificity, 90% (95% CI 87 to 92). It is not known if the bedside implementation of ALI "sniffer" will improve the adherence to evidence-based therapies and outcome of patients with ALI.

  18. Bilateral complex regional pain syndrome following spinal cord injury and bilateral calcaneus fracture

    Directory of Open Access Journals (Sweden)

    Ahmet Boyacı

    2013-09-01

    Full Text Available Complex regional pain syndrome (CRPS is a disease affectingone or more extremities, characterized by spontaneouspain, allodynia, hyperpathia and hyperalgesia.CRPS is separated into Type 1 and Type 2. CRPS whichdevelops after a nociceptive event is labeled as Type 1and when it develops following peripheral nerve damage,Type 2. Although the pathogenesis is not fully understood,peripheral and central sensitivity are held responsible.Bilateral lower extremity involvement is extremely rare.However, it should be borne in mind that it can develop intraumatic injuries which occur in more than one area anddiagnosis and commencement of a rehabilitation programshould be made in the early period. The case is presentedhere of bilateral Type 1 CRPS developing after incompletespinal cord injury and bilateral calcaneus fracture. JClin Exp Invest 2013; 4 (3: 360-363Key words: complex regional pain syndrome, calcaneusfracture, spinal cord injury

  19. Risk Factors for Post-treatment Complex Regional Pain Syndrome (CRPS)

    DEFF Research Database (Denmark)

    Petersen, Pelle B; Mikkelsen, Kim Lyngby; Lauritzen, Jes B

    2018-01-01

    OBJECTIVES: Complex regional pain syndrome is a challenging condition that includes a broad spectrum of sensory, autonomic, and motor features predominantly in extremities recovering from a trauma. Few large-scale studies have addressed occurrence of and factors associated with complex regional......, gender, initial diagnosis, treatment, and amount of compensation were extracted. Multivariate logistic regressions were performed to identify variables associated with approval of the claim. For carpal tunnel syndrome (CTS) patients, we registered whether symptoms were bilateral or unilateral...... normal neurophysiology. CONCLUSIONS: Female gender, surgical treatment, and treatment to the upper limb were risk factors. Elective surgery accounted for a large number of post-treatment CRPS patients. In CTS patients developing CRPS, normal neurophysiological examination findings were common...

  20. Sub-Symptomatic Aerobic Exercise for Patients with Post-Concussion Syndrome: A Critically Appraised Topic.

    Science.gov (United States)

    Ritter, Katrina G; Hussey, Matthew J; Valovich McLeod, Tamara C

    2017-09-27

    Clinical Scenario: Patients who experience prolonged concussion symptoms can be diagnosed with Post-Concussion Syndrome (PCS) when those symptoms persist past 4 weeks. Aerobic exercise protocols have been shown to be effective in improving physical and mental aspects of health. Emerging research suggests that aerobic exercise maybe useful as a treatment for PCS, where exercise allows patients to feel less isolated and more active during the recovery process. Is aerobic exercise more beneficial in reducing symptoms than current standard care in patients with prolonged symptoms or PCS lasting longer than 4 weeks? Summary of Key Findings: After a thorough literature search, 4 studies were selected relevant to the clinical question. Of the 4 studies, 1 was a randomized control trial and 3 were case series. All 4 studies investigate aerobic exercise protocol as treatment for PCS. 1-4 Three articles demonstrated a greater rate of symptom improvement from baseline assessment to follow-up after a controlled sub-symptomatic aerobic exercise program. 2-4 One study showed a decrease in symptoms in the aerobic exercise group compared to the full body stretching group. 1 Clinical Bottom Line: There is moderate evidence to support sub-symptomatic aerobic exercise as a treatment of PCS, therefore it should be considered as a clinical option for reducing PCS and prolonged concussion symptoms. A previously validated protocol, such as the Buffalo Concussion Treadmill Test, Balke Protocol, or Rating of Perceived Exertion (RPE) as mentioned in this critically appraised topic should be used to measure baseline values and treatment progression. Strength of Recommendation: Level C evidence exists that aerobic exercise protocol is more effective than the current standard of care in treating PCS.

  1. The sodium pentothal hypnosis interview with follow-up treatment for complex regional pain syndrome.

    Science.gov (United States)

    Simon, E P; Dahl, L F

    1999-08-01

    A patient who was unresponsive to multiple conservative medical treatments for complex regional pain syndrome was assessed using a novel approach--the sodium pentothal hypnosis interview. The interview suggested that his pain was centrally generated. The patient's pain symptoms resolved with hypnotherapeutic treatment. Indications for this procedure and implications for assessment and treatment are discussed. This case raises more questions than it answers, and leaves the reader to struggle with current difficulties in diagnostic decision-making.

  2. Hantavirus pulmonary syndrome (rio Mamore virus) in the peruvian Amazon region

    OpenAIRE

    Mamani, Enrique; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. biólogo.; García, María P.; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. tecnólogo médico.; Miraval, María L.; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. médico anátomo-patólogo.; Valencia, Pedro; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. Médico infectólogo.; Quino, Alberto H.; Hospital Regional de Loreto, Gobierno Regional de Loreto. Loreto, Perú. médico intensivista.; Álvarez, Carlos; Unidad de Análisis y Generación de Evidencias en Salud Pública. Instituto Nacional de Salud. Lima, Perú. médico anátomo-patólogo.; Donaires, Luis F.; Dirección Regional de Salud de Loreto, Gobierno Regional de Loreto. Loreto, Perú. médico epidemiólogo.

    2014-01-01

    Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analy...

  3. Normal regional brain iron concentration in restless legs syndrome measured by MRI

    Directory of Open Access Journals (Sweden)

    Susanne Knake

    2009-12-01

    Full Text Available Susanne Knake1, Johannes T Heverhagen2, Katja Menzler1, Boris Keil2, Wolfgang H Oertel1, Karin Stiasny-Kolster11Department of Neurology, Center of Nervous Diseases, 2Department of Radiology, Philipps University, Marburg, GermanyAbstract: Using a T2* gradient echo magnetic resonance imaging (MRI sequence, regional T2 signal intensity (SI values, a surrogate marker for T2 values, were determined in 12 regions of interest (substantia nigra, pallidum, caudate head, thalamus, occipital white matter, and frontal white matter bilaterally and in two reference regions (cerebrospinal fluid and bone in 12 patients suffering from moderate to severe idiopathic restless legs syndrome (RLS; mean age 58.5 ± 8.7 years for 12.1 ± 9.1 years and in 12 healthy control subjects (mean age 56.8 ± 10.6 years. Iron deposits shorten T2 relaxation times on T2-weighted MRI. We used regional T2* SI to estimate regional T2-values. A T2-change ratio was calculated for each region of interest relative to the reference regions. We did not find significant differences in any of the investigated brain regions. In addition, serum measures involved in iron metabolism did not correlate with T2 SI values. We could not replicate earlier findings describing reduced regional brain iron concentrations in patients with RLS. Our results do not support the view of substantially impaired regional brain iron in RLS.Keywords: restless legs syndrome, pathophysiology, iron, MRI, substantia nigra

  4. Chronic pain and evoked responses in the brain: A magnetoencephalographic study in Complex Regional Pain Syndrome I and II

    NARCIS (Netherlands)

    Theuvenet, P.J.

    2012-01-01

    Complex Regional Pain Syndrome (CRPS) type I and II are chronic pain syndromes with comparable symptoms, only in CRPS II a peripheral nerve injury is present. No objective tests are currently available to differentiate the two types which hampers diagnosis and treatment. Non-invasive brain imaging

  5. Identify fracture-critical regions inside the proximal femur using statistical parametric mapping

    Science.gov (United States)

    Li, Wenjun; Kornak, John; Harris, Tamara; Keyak, Joyce; Li, Caixia; Lu, Ying; Cheng, Xiaoguang; Lang, Thomas

    2009-01-01

    We identified regions inside the proximal femur that are most strongly associated with hip fracture. Bone densitometry based on such fracture-critical regions showed improved power in discriminating fracture patients from controls. Introduction Hip fractures typically occur in lateral falls, with focal mechanical failure of the sub-volumes of tissue in which the applied stress exceeds the strength. In this study, we describe a new methodology to identify proximal femoral tissue elements with highest association with hip fracture. We hypothesize that bone mineral density (BMD) measured in such sub-volumes discriminates hip fracture risk better than BMD in standard anatomic regions such as the femoral neck and trochanter. Materials and Methods We employed inter-subject registration to transform hip QCT images of 37 patients with hip fractures and 38 age-matched controls into a voxel-based statistical atlas. Within voxels, we performed t-tests between the two groups to identify the regions which differed most. We then randomly divided the 75 scans into a training set and a test set. From the training set, we derived a fracture-driven region of interest (ROI) based on association with fracture. In the test set, we measured BMD in this ROI to determine fracture discrimination efficacy using ROC analysis. Additionally, we compared the BMD distribution differences between the 29 patients with neck fractures and the 8 patients with trochanteric fractures. Results By evaluating fracture discrimination power based on ROC analysis, the fracture-driven ROI had an AUC (area under curve) of 0.92, while anatomic ROIs (including the entire proximal femur, the femoral neck, trochanter and their cortical and trabecular compartments) had AUC values between 0.78 and 0.87. We also observed that the neck fracture patients had lower BMD (p=0.014) in a small region near the femoral neck and the femoral head, and patients with trochanteric fractures had lower BMD in trochanteric regions

  6. Rare-Region-Induced Avoided Quantum Criticality in Disordered Three-Dimensional Dirac and Weyl Semimetals

    Directory of Open Access Journals (Sweden)

    J. H. Pixley

    2016-06-01

    Full Text Available We numerically study the effect of short-ranged potential disorder on massless noninteracting three-dimensional Dirac and Weyl fermions, with a focus on the question of the proposed (and extensively theoretically studied quantum critical point separating semimetal and diffusive-metal phases. We determine the properties of the eigenstates of the disordered Dirac Hamiltonian (H and exactly calculate the density of states (DOS near zero energy, using a combination of Lanczos on H^{2} and the kernel polynomial method on H. We establish the existence of two distinct types of low-energy eigenstates contributing to the disordered density of states in the weak-disorder semimetal regime. These are (i typical eigenstates that are well described by linearly dispersing perturbatively dressed Dirac states and (ii nonperturbative rare eigenstates that are weakly dispersive and quasilocalized in the real-space regions with the largest (and rarest local random potential. Using twisted boundary conditions, we are able to systematically find and study these two (essentially independent types of eigenstates. We find that the Dirac states contribute low-energy peaks in the finite-size DOS that arise from the clean eigenstates which shift and broaden in the presence of disorder. On the other hand, we establish that the rare quasilocalized eigenstates contribute a nonzero background DOS which is only weakly energy dependent near zero energy and is exponentially small at weak disorder. We also find that the expected semimetal to diffusive-metal quantum critical point is converted to an avoided quantum criticality that is “rounded out” by nonperturbative effects, with no signs of any singular behavior in the DOS at the energy of the clean Dirac point. However, the crossover effects of the avoided (or hidden criticality manifest themselves in a so-called quantum critical fan region away from the Dirac energy. We discuss the implications of our results for

  7. Are you better? A multi-centre study of patient-defined recovery from Complex Regional Pain Syndrome

    DEFF Research Database (Denmark)

    Llewellyn, A; McCabe, CS; Hibberd, Y

    2018-01-01

    Background: Complex Regional Pain Syndrome (CRPS) symptoms can significantly differ between patients, fluctuate over time, disappear or persist. This leads to problems in defining recovery and in evaluating the efficacy of therapeutic interventions. Objectives: To define recovery from the patient...

  8. Role of disorder in the multi-critical region of d-wave superconductivity and antiferromagnetism

    International Nuclear Information System (INIS)

    Yanase, Youichi; Ogata, Masao

    2007-01-01

    We investigate the disorder-induced microscopic inhomogeneity in the multi-critical region of d-wave superconductivity and antiferromagnetism on the basis of the microscopic t-t ' -U-V model. We find that a small amount of point disorder induces the nano-scale inhomogeneity of spin and superconducting fluctuations when the coherence length of superconductivity is remarkably short as in the under-doped cuprates. Then, the two fluctuations spatially segregate to avoid their competition. We show the remarkable electron-hole asymmetry in high-T c cuprates where the quite different spatial structure is expected in the electron-doped materials

  9. Critical region of a type II superconducting film near Hsub(c2): rational approximants

    International Nuclear Information System (INIS)

    Ruggeri, G.J.

    1979-01-01

    The high-temperature perturbative expansions for the thermal quantities of a type II superconducting film are extrapolated to the critical region near Hsub(c2) by means of new rational approximants of the Pade type. The new approximants are forced to reproduce the leading correction to the flux lattice contribution on the low-temperature side of the transition. Compared to those previously considered in the literature: (i) the mutual consistency of the approximants is improved; and (ii) they are nearer to the exact solution of the zero-dimensional Landau-Ginsburg model. (author)

  10. 57Fe Moessbauer studies on Ni-Mo system in the critical region

    International Nuclear Information System (INIS)

    Das, D.; Chintalapudi, S.N.; Mukhopadhyay, P.K.; Mookerjee, A.; Mukherjee, G.D.

    2001-01-01

    Disordered magnetic system NiMo is investigated in the critical region (Mo concentration 10 and 11 wt %) using Moessbauer spectroscopy as a local probe. 57 Co activity has been diffused in the alloy and is used at the source while stainless steel is used as standard absorber. Moessbauer spectrum of the alloy showed a sharp singlet at room temperature which indicates that 57 Co atoms have gone to the substitutional site. Below 200 K, Moessbauer spectra indicate complicated hyperfine interactions and more than one magnetic phase in the samples. Moessbauer results are corroborated by ac susceptibility, resistivity and positron annihilation Doppler broadening measurements. (author)

  11. Gain of chromosomal region 20q and loss of 18 discriminates between Lynch syndrome and familial colorectal cancer

    DEFF Research Database (Denmark)

    Therkildsen, Christina; Jönsson, Göran; Dominguez-Valentin, Mev

    2013-01-01

    Lynch syndrome and familial colorectal cancer type X, FCCTX, represent the two predominant colorectal cancer syndromes. Whereas Lynch syndrome is clinically and genetically well defined, the genetic cause of FCCTX is unknown and genomic differences between Lynch syndrome and FCCTX tumours...... are largely unknown. We applied array-based comparative genomic hybridisation to 23 colorectal cancers from FCCTX with comparison to 23 Lynch syndrome tumours and to 45 sporadic colorectal cancers. FCCTX tumours showed genomic complexity with frequent gains on chromosomes 20q, 19 and 17 and losses of 18, 8p...... and 15. Gain of genetic material in two separate regions encompassing, 20q12-13.12 and 20q13.2-13.32, was identified in 65% of the FCCTX tumours. Gain of material on chromosome 20q and loss on chromosome 18 significantly discriminated colorectal cancers associated with FCCTX from Lynch syndrome, which...

  12. Down's syndrome in the Lothian region of Scotland - 1978 to 1989

    International Nuclear Information System (INIS)

    Ramsay, C.N.; Ellis, P.M.; Zealley, H.

    1991-01-01

    A study of Down's syndrome in Lothian for the years 1978-1989 revealed a significantly higher than expected incidence of cases in 1987. The increased incidence was particularly significant among women aged 35 years or more. The increase could not be attributed to demographic changes on birth rates or to the age distribution of the women concerned. A temporal association between the cluster of cases and the events at Chernobyl in April 1986 was demonstrated. However, according to current understanding, there is no biologically plausible explanation to link the 2 events, given the reported levels of radioactive fallout in the Lothian region, resulting from the Chernobyl disaster. An explanation for the increased incidence of Down's syndrome in 1987 remains outstanding

  13. Particle acceleration in solar active regions being in the state of self-organized criticality.

    Science.gov (United States)

    Vlahos, Loukas

    We review the recent observational results on flare initiation and particle acceleration in solar active regions. Elaborating a statistical approach to describe the spatiotemporally intermittent electric field structures formed inside a flaring solar active region, we investigate the efficiency of such structures in accelerating charged particles (electrons and protons). The large-scale magnetic configuration in the solar atmosphere responds to the strong turbulent flows that convey perturbations across the active region by initiating avalanche-type processes. The resulting unstable structures correspond to small-scale dissipation regions hosting strong electric fields. Previous research on particle acceleration in strongly turbulent plasmas provides a general framework for addressing such a problem. This framework combines various electromagnetic field configurations obtained by magnetohydrodynamical (MHD) or cellular automata (CA) simulations, or by employing a statistical description of the field’s strength and configuration with test particle simulations. We work on data-driven 3D magnetic field extrapolations, based on a self-organized criticality models (SOC). A relativistic test-particle simulation traces each particle’s guiding center within these configurations. Using the simulated particle-energy distributions we test our results against observations, in the framework of the collisional thick target model (CTTM) of solar hard X-ray (HXR) emission and compare our results with the current observations.

  14. Critical issues in implementing low vision care in the Asia-Pacific region

    Directory of Open Access Journals (Sweden)

    Peggy Pei-Chia Chiang

    2012-01-01

    Full Text Available Two-thirds of the world′s population with low vision resides in the Asia-Pacific region. Provision of comprehensive low vision services is important to improve vision-related quality of life (QoL for people with this condition. This review outlines the critical issues and challenges facing the provision of low vision services in the Asia-Pacific region. The review offers possible strategies to tackle these issues and challenges facing service providers and policy makers in lieu of Vision 2020 strategies in this area. Pertinent findings from the global survey of low vision services and extensive ground work conducted in the region are used; in addition, a discussion on the availability of services, human resources and training, and funding and the future sustainability of low vision care will be covered. In summary, current issues and challenges facing the region are the lack of specific evidence-based data, access, appropriate equipment and facilities, human resources, funding, and sustainability. These issues are inextricably interlinked and thus cannot be addressed in isolation. The solutions proposed cover all areas of the VISION 2020 strategy that include service delivery, human resources, infrastructure and equipment, advocacy and partnership; and include provision of comprehensive care via vertical and horizontal integration; strengthening primary level care in the community; providing formal and informal training to enable task shifting and capacity building; and promoting strong government and private sector partnership to achieve long-term service financial sustainability.

  15. Endoscopic endonasal anatomy of superior orbital fissure and orbital apex regions: critical considerations for clinical applications.

    Science.gov (United States)

    Dallan, Iacopo; Castelnuovo, Paolo; de Notaris, Matteo; Sellari-Franceschini, Stefano; Lenzi, Riccardo; Turri-Zanoni, Mario; Battaglia, Paolo; Prats-Galino, Alberto

    2013-05-01

    The superior orbital fissure is a critical three-dimensional space connecting the middle cranial fossa and the orbit. From an endoscopic viewpoint, only the medial aspect has a clinical significance. It presents a critical relationship with the lateral sellar compartment, the pterygopalatine fossa and the middle cranial fossa. The connective tissue layers and neural and vascular structures of this region are described. The role of Muller's muscle is confirmed, and the utility of the maxillary and optic strut is outlined. Muller's muscle extends for the whole length of the inferior orbital fissure, passes over the maxillary strut and enters the superior orbital fissure, representing a critical surgical landmark. Dividing the tendon between the medial and inferior rectus muscle allows the identification of the main trunk of the oculomotor nerve, and a little laterally, it is usually possible to visualize the first part of the ophthalmic artery. Based on a better knowledge of anatomy, we trust that this area could be readily addressed in clinical situations requiring an extended approach in proximity of the orbital apex.

  16. Regional cerebral glucose metabolism in patients with alcoholic Korsakoff's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kessler, R.M.; Parker, E.S.; Clark, C.M.; Martin, P.R.; George, D.T.; Weingartner, H.; Sokoloff, L.; Ebert, M.H.; Mishkin, M.

    1985-05-01

    Seven alcoholic male subjects diagnosed as having Korsakoff's syndrome and eight age-matched male normal volunteers were studied with /sup 18/F 2-fluoro-2-deoxy-D-glucose (2/sup 18/FDG). All subjects were examined at rest with eyes covered in a quiet, darkened room. Serial plasma samples were obtained following injection of 4 to 5 mCi of 2/sup 18/FDG. Tomographic slices spaced at 10mm axial increments were obtained (in-plane resolution = 1.75 cm, axial resolution = 1.78 cm). Four planes were selected from each subject, and a total of 46 regions of interest were sampled and glucose metabolic rates for each region calculated. The mean glucose metalbolic rate for the 46 regions in the Korsakoff subjects was significantly lower than that in the normal controls (5.17 +- .43 versus 6.6 +- 1.31). A Q-component analysis, which examined each subject's regional rates relative to his mean rate, revealed two distinct patterns in the Korsakoff group. Glucose metabolism was significantly reduced in 37 of the 46 regions sampled. Reduced cerebral glucose metabolism in a nondemented group of subjects has not previously been reported. The reduction in cortical metabolism may be the result of damage to sub-cortical projecting systems. The differing patterns of cerebral metabolism in Korsakoff's syndrome suggests subgroups with differing neuropathology. Regions implicated in memory function, medial temporal, thalamic and medial prefrontal were among the regions reduced in metabolism.

  17. Scaled equation of state parameters for gases in the critical region

    Science.gov (United States)

    Sengers, J. M. H. L.; Greer, W. L.; Sengers, J. V.

    1976-01-01

    In the light of recent theoretical developments, the paper presents an accurate characterization of anomalous thermodynamic behavior of xenon, helium 4, helium 3, carbon dioxide, steam and oxygen in the critical region. This behavior is associated with long range fluctuations in the system and the physical properties depend primarily on a single variable, namely, the correlation length. A description of the thermodynamic behavior of fluids in terms of scaling laws is formulated, and the two successfully used scaled equations of state (NBS equation and Linear Model parametric equation) are compared. Methods for fitting both equations to experimental equation of state data are developed and formulated, and the optimum fit for each of the two scaled equations of the above gases are presented and the results are compared. By extending the experimental data for the above one-component fluids to partially miscible binary liquids, superfluid liquid helium, ferromagnets and solids exhibiting order-disorder transitions, the principle of universality is concluded. Finally by using this principle, the critical regions for nine additional fluids are described.

  18. Observation of unusual critical region behavior in the magnetic susceptibility of EuSe

    Science.gov (United States)

    Bykovetz, N.; Klein, J.; Lin, C. L.

    2018-05-01

    The Europium Chalcogenides (EuCh: EuO, EuS, EuSe, and EuTe) have been regarded as model examples of simple, cubic, Heisenberg exchange coupled magnetic systems, with a ferromagnetic nearest-neighbor exchange constant J1 and an antiferromagnetic next-nearest-neighbor constant J2. Unlike the other EuCh, EuSe exhibits a range of complex magnetic behaviors, the latter being attributed to EuSe being near the point where J2=-J1, where its magnetism appears to consist of nearly de-coupled 2D ferromagnetic sheets. Analysis of precision SQUID measurements of the magnetic susceptibility χ in EuSe showed that in the region from ˜Tc to ˜2Tc, a fit of the data to the critical equation χ = χ2Tc(T/Tc-1)-γ gives γ=2.0, an exponent not predicted by any current theory. Additionally, this fit predicts that Tc should be ˜0K. We tentatively interpret this by saying that in the paramagnetic region the system "thinks" EuSe should not order above T=0. Tc=0K is predicted by the Mermin-Wagner theorem (MW) for Heisenberg-coupled 2D magnetic systems, and we can show that when J2=-J1, MW can also be applied to the J1, J2 exchange model of the EuCh to give a rigorous Tc=0 prediction. Under 10 kbar applied pressure EuSe exhibits a different γ and fitted Tc. An additional, and rather strange, critical-region effect was discovered. The EuSe sample was found to exhibit a relaxation effect in a small range of temperatures, just above and just below the actual Tc of 4.7K, with time constants of up to 5 minutes. We cannot yet fully explain this observed macroscopic effect.

  19. The cld mutation: narrowing the critical chromosomal region and selecting candidate genes.

    Science.gov (United States)

    Péterfy, Miklós; Mao, Hui Z; Doolittle, Mark H

    2006-10-01

    Combined lipase deficiency (cld) is a recessive, lethal mutation specific to the tw73 haplotype on mouse Chromosome 17. While the cld mutation results in lipase proteins that are inactive, aggregated, and retained in the endoplasmic reticulum (ER), it maps separately from the lipase structural genes. We have narrowed the gene critical region by about 50% using the tw18 haplotype for deletion mapping and a recombinant chromosome used originally to map cld with respect to the phenotypic marker tf. The region now extends from 22 to 25.6 Mbp on the wild-type chromosome, currently containing 149 genes and 50 expressed sequence tags (ESTs). To identify the affected gene, we have selected candidates based on their known role in associated biological processes, cellular components, and molecular functions that best fit with the predicted function of the cld gene. A secondary approach was based on differences in mRNA levels between mutant (cld/cld) and unaffected (+/cld) cells. Using both approaches, we have identified seven functional candidates with an ER localization and/or an involvement in protein maturation and folding that could explain the lipase deficiency, and six expression candidates that exhibit large differences in mRNA levels between mutant and unaffected cells. Significantly, two genes were found to be candidates with regard to both function and expression, thus emerging as the strongest candidates for cld. We discuss the implications of our mapping results and our selection of candidates with respect to other genes, deletions, and mutations occurring in the cld critical region.

  20. A critical reappraisal of bilateral adrenalectomy for ACTH-dependent Cushing's syndrome.

    Science.gov (United States)

    Reincke, Martin; Ritzel, Katrin; Oßwald, Andrea; Berr, Christina; Stalla, Günter; Hallfeldt, Klaus; Reisch, Nicole; Schopohl, Jochen; Beuschlein, Felix

    2015-10-01

    Our aim was to review short- and long-term outcomes of patients treated with bilateral adrenalectomy (BADx) in ACTH-dependent Cushing's syndrome. We reviewed the literature and analysed our experience with 53 patients treated with BADx since 1990 in our institution. BADx is considered if ACTH-dependent Cushing's syndrome is refractory to other treatment modalities. In Cushing's disease (CD), BADx is mainly used as an ultima ratio after transsphenoidal surgery and medical therapies have failed. In these cases, the time span between the first diagnosis of CD and treatment with BADx is relatively long (median 44 months). In ectopic Cushing's syndrome, the time from diagnosis to BADx is shorter (median 2 months), and BADx is often performed as an emergency procedure because of life-threatening complications of severe hypercortisolism. In both situations, BADx is relatively safe (median surgical morbidity 15%; median surgical mortality 3%) and provides excellent control of hypercortisolism; Cushing's-associated signs and symptoms are rapidly corrected, and co-morbidities are stabilised. In CD, the quality of life following BADx is rapidly improving, and long-term mortality is low. Specific long-term complications include the development of adrenal crisis and Nelson's syndrome. In ectopic Cushing's syndrome, long-term mortality is high but is mostly dependent on the prognosis of the underlying malignant neuroendocrine tumour. BADx is a relatively safe and highly effective treatment, and it provides adequate control of long-term co-morbidities associated with hypercortisolism. © 2015 European Society of Endocrinology.

  1. Future temperature changes over the critical Belt and Road region based on CMIP5 models

    Directory of Open Access Journals (Sweden)

    Tian-Yun Dong

    2018-03-01

    Full Text Available Based on data of 22 models from the Coupled Model Inter-comparison Project Phase 5 (CMIP5, the performance of climate simulation is assessed and future changes under RCP2.6, RCP4.5 and RCP8.5 are projected over critical Belt and Road region. Compared with observations, the CMIP5 models simulate the linear trend and spatial distribution of the annual mean surface air temperature (SAT better in the north (NBR and south (SBR of the Belt and Road region. The trend of the 22-model ensemble mean (CMIP5 MME is 0.70/0.50 °C per 100 years from 1901 to 2005, and the observed trend is 1.11/0.77 °C per 100 years in the NBR/SBR region. After 1971, the relative error between CMIP5 MME and observations is 22%/15% in the NBR/SBR region. Seven/nine models are selected in the NBR/SBR to project future SAT changes under three RCP scenarios. For 2081–2100, warming in the NBR/SBR is projected to be (1.16 ± 0.29/(0.72 ± 0.32 °C, (2.41 ± 0.54/(1.55 ± 0.44 °C, and (5.23 ± 1.02/(3.33 ± 0.65 °C for RCP2.6, RCP4.5, and RCP8.5, respectively. Under the RCP scenarios, the NBR region shows greater warming than the SBR region. The most significant warming is expected in Kazakhstan and the northern part of the SBR. The associated uncertainty generally increases with time under the three RCP scenarios. Furthermore, increases in warming over the Belt and Road region are more remarkable under higher-emission scenarios than lower-emission ones. Keywords: CMIP5 models, The Belt and Road region, Temperature projection, RCPs

  2. Regional cerebral blood flow characteristics of the Sturge-Weber syndrome

    International Nuclear Information System (INIS)

    Riela, A.R.; Stump, D.A.; Roach, E.S.; McLean, W.T. Jr.; Garcia, J.C.

    1985-01-01

    Four patients with the Sturge-Weber syndrome were studied using the non-invasive Xenon-133 inhalation technique. All four patients had decreased regional cerebral blood flow in the area of their lesion, and in two patients who were subsequently tested with 5% carbon dioxide inhalation, impaired vasomotor reactivity was documented. Diminished regional cerebral blood flow is consistent with previously described nuclide flow studies which demonstrated a delay in the initial perfusion blush in the region of the abnormal vasculature. The focal decrease in blood flow was greatest in the most severely affected patient, but was also prominent in the two younger patients, both of whom have excellent neurologic function. These studies suggest that localized decrease in blood flow and vasomotor dysfunction in Sturge-Weber syndrome can precede the occurrence of severe neurologic impairment and extensive cerebral atrophy and possibly be a major contributing factor in progressive dysfunction. A secondary observation was that the blood flow in the unaffected hemisphere was significantly greater in two children compared to the two adults and was similar to the age-related differences reported for normal children and adults

  3. Clinical and epidemiological features of hemorrhagic fever with renal syndrome course in Perm region

    Directory of Open Access Journals (Sweden)

    M. V. Ivanova

    2011-01-01

    Full Text Available The results of retrospective analysis of hemorrhagic fever with renal syndrome (HFRS incidence in the Perm region for 1995–2009 are shown in the article. There are highly active natural foci of infection on the territory of the region, mainly in the subzone of mixed coniferous-deciduous forests. Factors supporting the morbidity are high numbers of small mammals, in particular, the bank vole, with a large circulation of the HFRS virus and active population visit of natural foci in summer-autumn period. Analysis of clinical features of HFRS in 338 patients living in the Perm region, revealed some peculiarities of its course. Among surveyed contingent men (72,4% prevailed. Most of the patients (80,4% were of working age (16 to 60 years. More frequently mild and moderate forms (92,8% of HFRS were observed. A typical picture of the disease with the development of all specific syndromes occurred mainly in patients with severe and moderate forms. Mild cases occurred blurry in the absence of pathognomonic symptoms. Notable was the frequent lesion of liver, with the development of acute anicteric hepatitis (51,2%. Among the complications dominated: pneumonia (4,7% and acute renal  ailure (4,4%. Manifestations of DIC syndrome with recurrent bleeding were registered in 0,6% of patients, toxic shock – at 0,3%. Case fatality rate was 0,6%.

  4. [Mitochondrial and microcirculatory distress syndrome in the critical patient. Therapeutic implications].

    Science.gov (United States)

    Navarrete, M L; Cerdeño, M C; Serra, M C; Conejero, R

    2013-10-01

    Mitochondrial and microcirculatory distress syndrome (MMDS) can occur during systemic inflammatory response syndrome (SIRS), and is characterized by cytopathic tissue hypoxia uncorrected by oxygen transport optimization, and associated with an acquired defect in the use of oxygen and energy production in mitochondria, leading to multiple organ dysfunction (MOD). We examine the pathogenesis of MMDS, new diagnostic methods, and recent therapeutic approaches adapted to each of the three phases in the evolution of the syndrome. In the initial phase, the aim is prevention and early reversal of mitochondrial dysfunction. Once the latter is established, the aim is to restore flow of the electron chain, mitochondrial respiration, and to avoid cellular energy collapse. Finally, in the third (resolution) stage, treatment should focus on stimulating mitochondrial biogenesis and the repair or replacement of damaged mitochondria. Copyright © 2012 Elsevier España, S.L. and SEMICYUC. All rights reserved.

  5. Critical Parameters and Critical-Region (p,ρ ,T) Data of trans-1,1,1,3-Tetrafluorobut-2-ene [HFO-1354mzy(E)

    Science.gov (United States)

    Kimura, Takeru; Kayukawa, Yohei; Miyamoto, Hiroyuki; Saito, Kiyoshi

    2017-08-01

    This study presents the experimental measurement of the pρ T properties and critical parameters of a low GWP type refrigerant, trans-1,1,1,3-Tetrafluorobut-2-ene (HFO-1354mzy(E)). The sample purity of the substance was 99 area %. p ρ T property measurements and visual observations of the meniscus of HFO-1354mzy(E) were carried out using a metal-bellows volumometer with an optical cell. The critical temperature was determined by observation of the critical opalescence. The critical pressure and critical density were determined as the inflection point of the isothermal p ρ T property data at the critical temperature. For more precise clarification of the thermodynamic surface in the vicinity of the critical point, additional p ρ T property measurements were carried out on three isotherms in the supercritical region. The expanded uncertainties (k = 2) in the temperature, pressure, and density measurements were estimated to be less than 3 mK, 1.2 kPa, and 0.32 \\hbox {kg} \\cdot \\hbox {m}^{-3}, respectively. The expanded uncertainties of the critical parameters were estimated to be less than 13 mK, 1.4 kPa, and 2.3 \\hbox {kg} \\cdot \\hbox {m}^{-3}, respectively. These values are the first reported for HFO-1354mzy(E) and are necessary for the development of its equation of state in the near future.

  6. Critical Magnetic Field Strengths for Unipolar Solar Coronal Plumes In Quiet Regions and Coronal Holes?

    Science.gov (United States)

    Avallone, Ellis; Tiwari, Sanjiv K.; Panesar, Navdeep K.; Moore, Ronald L.; Winebarger, Amy

    2017-01-01

    Coronal plumes are bright magnetic funnels that are found in quiet regions and coronal holes that extend high into the solar corona whose lifetimes can last from hours to days. The heating processes that make plumes bright involve the magnetic field at the base of the plume, but their intricacies remain mysterious. Raouafi et al. (2014) infer from observation that plume heating is a consequence of magnetic reconnection at the base, whereas Wang et al. (2016) infer that plume heating is a result of convergence of the magnetic flux at the plume's base, or base flux. Both papers suggest that the base flux in their plumes is of mixed polarity, but do not quantitatively measure the base flux or consider whether a critical magnetic field strength is required for plume production. To investigate the magnetic origins of plume heating, we track plume luminosity in the 171 Å wavelength as well as the abundance and strength of the base flux over the lifetimes of six unipolar coronal plumes. Of these, three are in coronal holes and three are in quiet regions. For this sample, we find that plume heating is triggered when convergence of the base flux surpasses a field strength of approximately 300 - 500 Gauss, and that the luminosity of both quiet region and coronal hole plumes respond similarly to the strength of the magnetic field in the base.

  7. Complex Regional Pain Syndrome (CRPS/RSD and Neuropathic Pain: Role of Intravenous Bisphosphonates as Analgesics

    Directory of Open Access Journals (Sweden)

    Jennifer Yanow

    2008-01-01

    Full Text Available Neuropathic pain is a sequela of dysfunction, injuries, or diseases of the peripheral and/or central nervous system pain pathways, which has historically been extremely difficult to treat. Complex regional pain syndrome (CRPS types 1 and 2 are neuropathic pain conditions that have a long history in the medical literature but whose pathophysiology remains elusive and whose available treatment options remain few. While an exact animal model for CRPS doesn't yet exist, there are several animal models of neuropathic pain that develop behaviors of hypersensitivity, one of the hallmark signs of neuropathic pain in humans.

  8. Inflammatory arthritis mimicking Complex Regional Pain Syndrome (CRPS) in a child: A case report.

    Science.gov (United States)

    Egilmez, Zeliha; Turgut, Selin Turan; Icagasioglu, Afitap; Bicakci, Irem

    2016-01-01

    Joint complaints in childhood are seen frequently and differential diagnosis can be difficult. Juvenile idiopathic arthritis (JIA) is the most common rheumatological disease of childhood. It involves peripheral joint arthritis, chronic synovitis, and extra-articular manifestations. Accurate diagnosis can take a long time and sometimes multiple diagnoses are used while following the patient until a final diagnosis can be reached. Arthritis may be triggered by trauma and confused with other diseases like complex regional pain syndrome (CRPS), in which trauma plays a role in the etiology. In the present case, ankle pain in an 8-year-old girl was misdiagnosed as CRPS.

  9. Clinical features and pathophysiology of Complex Regional Pain Syndrome – current state of the art

    Science.gov (United States)

    Marinus, Johan; Moseley, G. Lorimer; Birklein, Frank; Baron, Ralf; Maihöfner, Christian; Kingery, Wade S.; van Hilten, Jacobus J.

    2017-01-01

    That a minor injury can trigger a complex regional pain syndrome (CRPS) - multiple system dysfunction, severe and often chronic pain and disability - has fascinated scientists and perplexed clinicians for decades. However, substantial advances across several medical disciplines have recently increased our understanding of CRPS. Compelling evidence implicates biological pathways that underlie aberrant inflammation, vasomotor dysfunction, and maladaptive neuroplasticity in the clinical features of CRPS. Collectively, the evidence points to CRPS being a multifactorial disorder that is associated with an aberrant host response to tissue injury. Varying susceptibility to perturbed regulation of any of the underlying biological pathways probably accounts for the clinical heterogeneity of CRPS. PMID:21683929

  10. Radiofrequency ablation of stellate ganglion in a patient with complex regional pain syndrome

    Directory of Open Access Journals (Sweden)

    Chinmoy Roy

    2014-01-01

    Full Text Available Complex regional pain syndrome (CRPS is characterized by a combination of sensory, motor, vasomotor, pseudomotor dysfunctions and trophic signs. We describe the use of radiofrequency (RF ablation of Stellate ganglion (SG under fluoroscopy, for long-term suppression of sympathetic nervous system, in a patient having CRPS-not otherwise specified. Although the effects of thermal RF neurolysis may be partial or temporary, they may promote better conditions toward rehabilitation. The beneficial effect obtained by the RF neurolysis of SG in this particular patient strongly advocates the use of this mode of therapy in patients with CRPS.

  11. Bilateral Complex Regional Pain Syndrome after Fracture of Bilateral Tibia and Fibula

    Directory of Open Access Journals (Sweden)

    Senem Şaş

    2017-04-01

    Full Text Available Complex regional syndrome type 1 (CRPS-1 is a painful clinical condition. It occurs after a painful event and characterized by allodynia, hyperalgesia, edema, abnormalities in skin blood flow and abnormal sudomotor activity. When CRPS-1 is associated with nerve injury, it is defined as CRPS-2. Central and peripheral theory are responsible in etiopathogenesis of CRPS-1. Generally it occurs in the injured limb. But, it may ocur in the opposite extremities. In this article, we present a case developing bilateral CRPS-1 after bilateral tibia and fibula fracture by reviewing current literatüre.

  12. Imaging of Posttraumatic Arthritis, Avascular Necrosis, Septic Arthritis, Complex Regional Pain Syndrome, and Cancer Mimicking Arthritis.

    Science.gov (United States)

    Rupasov, Andrey; Cain, Usa; Montoya, Simone; Blickman, Johan G

    2017-09-01

    This article focuses on the imaging of 5 discrete entities with a common end result of disability: posttraumatic arthritis, a common form of secondary osteoarthritis that results from a prior insult to the joint; avascular necrosis, a disease of impaired osseous blood flow, leading to cellular death and subsequent osseous collapse; septic arthritis, an infectious process leading to destructive changes within the joint; complex regional pain syndrome, a chronic limb-confined painful condition arising after injury; and cases of cancer mimicking arthritis, in which the initial findings seem to represent arthritis, despite a more insidious cause. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Emotional intelligence moderates the relationship between regional gray matter volume in the bilateral temporal pole and critical thinking disposition.

    Science.gov (United States)

    Yao, Xiaonan; Yuan, Shuge; Yang, Wenjing; Chen, Qunlin; Wei, Dongtao; Hou, Yuling; Zhang, Lijie; Qiu, Jiang; Yang, Dong

    2018-04-01

    Critical thinking enables people to form sound beliefs and provides a basis for emotional life. Research has indicated that individuals with better critical thinking disposition can better recognize and regulate their emotions, though the neuroanatomical mechanisms involved in this process remain to be elucidated. Further, the influence of emotional intelligence on the relationship between brain structure and critical thinking disposition has not been examined. The present study utilized voxel-based morphometry (VBM) to investigate the neural structures underlying critical thinking disposition in a large sample of college students (N = 296). Regional gray matter volume (rGMV) in the bilateral temporal pole, which reflects an individual's ability to process social and emotional information, was negatively correlated with critical thinking disposition. In addition, rGMV in bilateral para hippocampal regions -regions involved in contextual association/emotional regulation-exhibited negative correlation with critical thinking disposition. Further analysis revealed that emotional intelligence moderated the relationship between rGMV of the temporal pole and critical thinking disposition. Specifically, critical thinking disposition was associated with decreased GMV of the temporal pole for individuals who have relatively higher emotional intelligence rather than lower emotional intelligence. The results of the present study indicate that people who have higher emotional intelligence exhibit more effective and automatic processing of emotional information and tend to be strong critical thinkers.

  14. Critical Involvement of Macrophage Infiltration in the Development of Sjogren's Syndrome-Associated Dry Eye

    NARCIS (Netherlands)

    Zhou, D.; Chen, Y.T.; Chen, F.L.; Gallup, M.; Vijmasi, T.; Bahrami, A.F.; Noble, L.B.; van Rooijen, N.; McNamara, N.A.

    2012-01-01

    Lymphocytic infiltration of the lacrimal gland and ocular surface in autoimmune diseases such as Sjögren's syndrome (SS) causes an aqueous-deficient dry eye that is associated with significant morbidity. Previous studies from our laboratory and others have established autoimmune regulator

  15. Hydrogeochemistry of prairie pothole region wetlands: Role of long-term critical zone processes

    Science.gov (United States)

    Goldhaber, Martin B.; Mills, Christopher T.; Morrison, Jean M.; Stricker, Craig A.; Mushet, David M.; LaBaugh, James W.

    2014-01-01

    This study addresses the geologic and hydrogeochemical processes operating at a range of scales within the prairie pothole region (PPR). The PPR is a 750,000 km2portion of north central North America that hosts millions of small wetlands known to be critical habitat for waterfowl and other wildlife. At a local scale, we characterized the geochemical evolution of the 92-ha Cottonwood Lake study area (CWLSA), located in North Dakota, USA. Critical zone processes are the long-term determinant of wetland water and groundwater geochemistry via the interaction of oxygenated groundwater with pyrite in the underlying glacial till. Pyrite oxidation produced a brown, iron oxide-bearing surface layer locally over 13 m thick and an estimated minimum of 1.3 × 1010 g sulfate (SO42 −) at CWLSA. We show that the majority of this SO42− now resides in solid-phase gypsum (CaSO4•2H2O) and gypsum-saturated groundwater.

  16. Effect of regional citrate anticoagulation on critical patients with continuous renal replacement therapy

    Directory of Open Access Journals (Sweden)

    Li-Li You

    2016-12-01

    Full Text Available Objective: To investigate the efficacy and safety of regional citrate anticoagulation (RCA in continuous renal replacement therapy (CRRT for critical patients. Methods: A total of 83 critical patients need CRRT in the intensive care units of our hospital from July 2012 to June 2016 were recruited in the study, and the patients were divided into two groups randomly, the patients in observation group received the RCA treatment, and the patients in control group received traditional low molecular heparin anticoagulation. The difference of safety indicators, biochemical indicators, extracorporeal circulation blood coagulation condition and complications in patients were determined between two groups. Results: Compared with control group, the patients in observation group had an elevated level of iCa2+, the level of chloride ion reduced, the use time of filter increased, the bleeding cases reduced, the concentrations of urea nitrogen, creatinine TNF-α , IL-1β, IL-8 and NO were all significantly downregulated, the data have a significant difference (P < 0.05. Conclusions: RCA is a safe and effective method for CRRT in patients with a high risk of bleeding.

  17. Diagnosis and fine localization of deletion region in Wolf-Hirschhorn syndrome patients.

    Science.gov (United States)

    Ji, Tao-Yun; Chia, David; Wang, Jing-Min; Wu, Ye; Li, Jie; Xiao, Jing; Jiang, Yu-Wu

    2010-07-01

    Wolf-Hirschhorn syndrome (WHS) results from the partial deletion of 4p. This study aimed to identify and fine map the chromosome deletion regions of Chinese children with Wolf-Hirschhorn syndrome among the developmental delay/mental retardation (DD/MR) patients. We analyzed the relationship of phenotype and genotype. Inclusion criteria were: moderate to severe DD/MR, no definite perinatal brain injury, and no trauma, toxication, hypoxia, infection of central nervous system; routine karyotyping was normal, no evidence of typical inherited metabolic disorder or specific neurodegenerative disorders from cranial neuro-imaging and blood/urinary metabolic diseases screening; no mutation of FMR1 in male patients, no typical clinical manifestation of Rett syndrome in female patients. Multiplex ligation-dependent probe amplification (MLPA) and Affymetrix genome-wide human SNP array 6.0 assays were applied to accurately define the exact size of subtelomeric aberration region of four WHS patients. All four WHS patients presented with severe DD, hypotonia and microcephaly, failure to thrive, 3/4 patients with typical facial features and seizures, 2/4 patients with congenital heart defects and cleft lip/palate, 1/4 patients with other malformations. The length of the deletions ranged from 3.3 Mb to 9.8 Mb. Two of four patients had "classic" WHS, 1/4 patients had "mild"-to-"classic" WHS, and 1/4 patients had "mild" WHS. WHS patients in China appear to be consistent with those previously reported. The prevalence of signs and symptoms, distribution of cases between "mild" and "classic" WHS, and the correlation between length of deletion and severity of disease of these patients were all similar to those of the patients from other populations.

  18. Differential Efficacy of Ketamine in the Acute versus Chronic Stages of Complex Regional Pain Syndrome in Mice

    Science.gov (United States)

    Tajerian, Maral; Leu, David; Yang, Phillip; Huang, Ting Ting; Kingery, Wade S; Clark, J David

    2015-01-01

    Background Complex regional pain syndrome (CRPS) is a painful, disabling and often chronic condition, where many patients transition from an acute phase with prominent peripheral neurogenic inflammation to a chronic phase with evident central nervous system (CNS) changes. Ketamine is a centrally-acting agent believed to work through blockade of N-methyl-D-aspartate (NMDA) receptors and is being increasingly used for the treatment of refractory CRPS, although the basis for the drug’s effects and efficacy at different stages of the syndrome remain unclear. Methods We used a mouse model of CRPS (n=8–12/group) involving tibia fracture/cast immobilization to test the efficacy of ketamine (2 mg/kg/day; 7 days) or vehicle infusion during acute (3weeks [3w] post-fracture) and chronic (7w post-fracture) stages. Results Acute phase fracture mice displayed elevated limb temperature, edema and nociceptive sensitization that were not reduced by ketamine. Fracture mice treated with ketamine during the chronic phase showed reduced nociceptive sensitization that persisted beyond completion of the infusion. During this chronic phase, ketamine also reduced latent nociceptive sensitization and improved motor function at 18 weeks post-fracture. No side effects of the infusions were identified. These behavioral changes were associated with altered spinal astrocyte activation and expression of pain-related proteins including NMDA receptor 2b (NR2b), Ca2+/calmodulin-dependent protein kinase ii (CaMK2), and brain-derived neurotrophic factor (BNDF). Conclusions Collectively, these results demonstrate that ketamine is efficacious in the chronic, but not acute stages of CRPS, suggesting that the centrally-acting drug is relatively ineffective in early CRPS when peripheral mechanisms are more critical for supporting nociceptive sensitization. PMID:26492479

  19. Fear of pain in children and adolescents with neuropathic pain and complex regional pain syndrome.

    Science.gov (United States)

    Simons, Laura E

    2016-02-01

    A significant proportion of children and adolescents with chronic pain endorse elevated pain-related fear. Pain-related fear is associated with high levels of disability, depressive symptoms, and school impairment. Because of faulty nerve signaling, individuals with neuropathic pain and complex regional pain syndrome may be more prone to develop pain-related fear as they avoid use of and neglect the affected body area(s), resulting in exacerbated symptoms, muscle atrophy, maintenance of pain signaling, and ongoing pain-related disability. Not surprisingly, effective treatments for elevated pain-related fears involve exposure to previously avoided activities to downregulate incorrect pain signaling. In the context of intensive interdisciplinary pain treatment of youth with neuropathic pain, decreasing pain-related fear is associated with improved physical and psychological functioning, whereas high initial pain-related fear is a risk factor for less treatment responsiveness. An innovative approach to targeting pain-related fear and evidence of a neural response to treatment involving decoupling of the amygdala with key fear circuits in youth with complex regional pain syndrome suggest breakthroughs in our ability to ameliorate these issues.

  20. Effects of A Combined Treatment Protocol in Chronic Regional Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Ali Asghar Jameh-Bozorgi

    2011-01-01

    Full Text Available Objective: Chronic regional pain syndrome (CRPS is one of the most important and worst types of peripheral nervous system, especially in upper extremity. The aim of this study was determination of the effect of a combined rehabilitation program in the treatment of patients with CRPS type I. Materials & Methods: In this quasi-experimental and before-after study, 20 patients with chronic regional pain syndrome were selected simply and their pain, range of motion, edema and muscular strength were examined and recorded before intervention. Then, patients under went a combined treatment programs included some modalities from physical and occupation therapy. Patients attended at clinic for 20 therapeutic sessions with one day intervals. Finally, data were analyzed using paired–t test. Results: Post operatively, pain and edema were decreased and range of motion and grip strength was increased significantly (P>0.05. Conclusion: Current study demonstrated that early and combined physical and occupational therapy efficient in the treatment of patients suffering from CRPS type I. This combined program can relieve pain and edema and increase ROM and grip strength.

  1. The N-terminal, polybasic region is critical for prion protein neuroprotective activity.

    Directory of Open Access Journals (Sweden)

    Jessie A Turnbaugh

    Full Text Available Several lines of evidence suggest that the normal form of the prion protein, PrP(C, exerts a neuroprotective activity against cellular stress or toxicity. One of the clearest examples of such activity is the ability of wild-type PrP(C to suppress the spontaneous neurodegenerative phenotype of transgenic mice expressing a deleted form of PrP (Δ32-134, called F35. To define domains of PrP involved in its neuroprotective activity, we have analyzed the ability of several deletion mutants of PrP (Δ23-31, Δ23-111, and Δ23-134 to rescue the phenotype of Tg(F35 mice. Surprisingly, all of these mutants displayed greatly diminished rescue activity, although Δ23-31 PrP partially suppressed neuronal loss when expressed at very high levels. Our results pinpoint the N-terminal, polybasic domain as a critical determinant of PrP(C neuroprotective activity, and suggest that identification of molecules interacting with this region will provide important clues regarding the normal function of the protein. Small molecule ligands targeting this region may also represent useful therapeutic agents for treatment of prion diseases.

  2. Coupled core criticality calculations with control rods located in the central reflector region

    Energy Technology Data Exchange (ETDEWEB)

    Sobhy, M [Reactor depatrment, nuclear research center, Inshaas (Egypt)

    1995-10-01

    The reactivity of a coupled core is controlled by a set of control rods distributed in the central reflector region. The reactor contains two compact cores cooled and moderated by light water. Control rods are designed to have reactivity worths sufficient to start, control and shutdown the coupled system. Each core in a coupled system is in subcritical conditions without any absorber then each core needs to the other core to fulfill nuclear chain reaction and to approach the criticality. In this case, each core is considered clean which is suitable for research reactor with low flux disturbance and better neutron economy, in addition to the advantage of disappearing the cut corner fuel baskets. This facilitate the in core fuel management with identical fuel baskets. Hot spots will disappear. This leads to a good heat transfer process. the excess reactivity and the shutdown margin are calculated for some of reflector as coupling region gives sufficient area for coupled core are calculated cost. The fluctuations of reactivity for coupled core are calculated by noise analysis technique and compared with that for rode core. The results show low reactivity perturbation associated with coupled core.

  3. The Responsive Amygdala: Treatment-induced Alterations in Functional Connectivity in Pediatric Complex Regional Pain Syndrome

    Science.gov (United States)

    Simons, LE; Pielech, M; Erpelding, N; Linnman, C; Moulton, E; Sava, S; Lebel, A; Serrano, P; Sethna, N; Berde, C; Becerra, L; Borsook, D

    2014-01-01

    The amygdala is a key brain region with efferent and afferent neural connections that involve complex behaviors such as pain, reward, fear and anxiety. This study evaluated resting state functional connectivity of the amygdala with cortical and subcortical regions in a group of chronic pain patients (pediatric complex regional pain syndrome) with age-gender matched controls before and after intensive physical-biobehavioral pain treatment. Our main findings include (1) enhanced functional connectivity from the amygdala to multiple cortical, subcortical, and cerebellar regions in patients compared to controls, with differences predominantly in the left amygdala in the pre-treated condition (disease state); (2) dampened hyperconnectivity from the left amygdala to the motor cortex, parietal lobe, and cingulate cortex after intensive pain rehabilitation treatment within patients with nominal differences observed among healthy controls from Time 1 to Time 2 (treatment effects); (3) functional connectivity to several regions key to fear circuitry (prefrontal cortex, bilateral middle temporal lobe, bilateral cingulate, hippocampus) correlated with higher pain-related fear scores and (4) decreases in pain-related fear associated with decreased connectivity between the amygdala and the motor and somatosensory cortex, cingulate, and frontal areas. Our data suggest that there are rapid changes in amygdala connectivity following an aggressive treatment program in children with chronic pain and intrinsic amygdala functional connectivity activity serving as a potential indicator of treatment response. PMID:24861582

  4. Differences in economic development in rural regions of advanced countries: an overview and critical analysis of theories

    NARCIS (Netherlands)

    Terluin, I.J.

    2003-01-01

    This article provides an overview and critical analysis of theories on economic development in rural regions in advanced countries. For this purpose, we have consulted literature in regional economics and the multidisciplinary field of rural studies. In order to analyse to which extent these

  5. [Prevalence and Co-prevalence of Complex Regional Pain Syndrome (CRPS) and Carpal Tunnel Syndrome (CTS) in Hand Rehabilitation].

    Science.gov (United States)

    Neubrech, F; Gentzsch, T; Kotsougiani, D; Bickert, B; Kneser, U; Harhaus, L

    2016-06-01

    In the current literature, there are reports of associations between complex regional pain syndromes (CRPS) and carpal tunnel syndromes (CTS). The aim of this study was to determine the prevalence of both disease patterns in hand rehabilitation patients and to investigate whether there is a correlation between CTS and CRPS. Furthermore, differences in the healing process of patients with and without additional CTS, and the effectiveness of the rehabilitative therapy for both diseases, were investigated. The computerised medical records of 791 patients in the years 2009-2015 who had been in hand rehabilitation were retrospectively analysed. At the beginning and end of rehabilitation, measurements were made of pain by visual analogue scales (VAS, 0-10), grip strength and finger mobility (mean distance from finger pulp to palmar D2-D5). The clinical course was statistically analysed. CRPS diagnosis was confirmed clinically by a pain therapist, CTS diagnosis was confirmed by neurological and neurophysiological examination. Surgical therapy was performed despite CRPS diagnosis. The prevalence of CRPS was 161/1000 and of CTS 62/1000; the co-prevalence of the 2 diagnoses was 24/1000 (pCRPS group, after a mean of 8 (1-21) weeks of rehabilitative therapy, mean pain was reduced from 5 (1-10) to 3 (0-9), grip strength improved from 10 (0-39)kg to 18.5 (2.5-45.5)kg and finger mobility increased from 2.9 (0-7.6)cm to 1.8 (0-7.8)cm. In the CRPS+CTS group, after a mean of 6.8 (3-23) weeks of rehabilitative therapy, mean pain was reduced from 5 (0-8) to 2.6 (0-5), grip strength improved from 9.7 (2.4-25.5)kg to 17.4 (0.9-47.4)kg and finger mobility increased from 2.7 (0-5.3)cm to 1.7 (0-5.3)cm. Improvement over the period of rehabilitation was significant in both groups, though the period of therapy was significantly shorter in the CRPS+CTS group. CRPS and CTS are often associated. Rehabilitative therapy was effective for CRPS- and CRPS+CTS patients. © Georg Thieme Verlag KG

  6. Parental imprinting of human chromosome region 11p15.3-pter involved in the Beckwith-Wiedemann syndrome and various human neoplasia

    NARCIS (Netherlands)

    Mannens, M.; Hoovers, J. M.; Redeker, E.; Verjaal, M.; Feinberg, A. P.; Little, P.; Boavida, M.; Coad, N.; Steenman, M.; Bliek, J.

    1994-01-01

    Cytogenetic and DNA analyses of patients with the Beckwith-Wiedemann syndrome (BWS) enabled us to refine the localization of the syndrome at 11p15.3-pter to two distinct regions. One chromosome region (BWSCR1) is near the insulin (INS) and insulin-like growth factor 2 (IGF2) genes. The other region

  7. The left fusiform gyrus is a critical region contributing to the core behavioral profile of semantic dementia

    Directory of Open Access Journals (Sweden)

    Junhua eDing

    2016-05-01

    Full Text Available Given that extensive cerebral regions are co-atrophic in semantic dementia (SD, it is not yet known which critical regions (SD-semantic-critical regions are really responsible for the semantic deficits of SD. To identify the SD-semantic-critical regions, we explored the relationship between the degree of cerebral atrophy in the whole brain and the severity of semantic deficits in 19 individuals with SD. We found that the gray matter volumes of two regions [left fusiform gyrus (lFFG and left parahippocampal gyrus (lPHG] significantly correlated with the semantic scores of patients with SD. Importantly, the effects of the lFFG remained significant after controlling for the gray matter volumes of the lPHG. Moreover, the effects of the region could not be accounted for by the total gray matter volume, general cognitive ability, laterality of brain atrophy, or control task performance. We further observed that each atrophic portion of the lFFG along the anterior-posterior axis might dedicate to the loss of semantic functions in SD. These results reveal that the lFFG could be a critical region contributing to the semantic deficits of SD.

  8. Complex regional pain syndrome (CRPS) type I: historical perspective and critical issues.

    Science.gov (United States)

    Iolascon, Giovanni; de Sire, Alessandro; Moretti, Antimo; Gimigliano, Francesca

    2015-01-01

    The history of algodystrophy is controversial and its denomination has changed significantly over time. Silas Weir Mitchell described several cases of causalgia due to gunshot wounds that occurred during the American Civil War, increasing knowledge about this clinical condition. A later key milestone in the history of CRPS is tied to the name of Paul Sudeck that, using X-ray examinations, described findings of bone atrophy following a traumatic event or infection of the upper limb. The most widely accepted pathogenic hypothesis, proposed by Rene Leriche, supported a key role of the sympathetic nervous system in the onset of the typical clinical picture of the disease, which was thus defined as "reflex sympathetic dystrophy". In the 50s John J. Bonica proposed a staging of CRPS. In a consensus conference held in Budapest in 2003, it was proposed a new classification system that included the presence of at least two clinical signs included in the four categories and at least three symptoms in its four categories. There have been other classification systems proposed for the diagnosis of CRPS, such as Veldman diagnostic criteria based on the presence of at least 4 signs and symptoms of the disease associated with a worsening of the same following the use of the limb and their location in the same area distal to the one that suffered the injury. On the other hand, the Atkins diagnostic criteria are much more objective than those proposed by IASP and are specifically applicable to an orthopaedic context. However, current classification systems and related criteria proposed to make a diagnosis of CRPS, do not include instrumental evaluations and imaging, but rely solely on clinical findings. This approach does not allow an optimal disease staging especially in orthopaedics.

  9. Impaired insula functional connectivity associated with persistent pain perception in patients with complex regional pain syndrome

    Science.gov (United States)

    Jang, Joon Hwan; Lee, Do-Hyeong; Lee, Kyung-Jun; Lee, Won Joon; Moon, Jee Youn; Kim, Yong Chul

    2017-01-01

    Given that the insula plays a contributory role in the perception of chronic pain, we examined the resting-state functional connectivity between the insular cortex and other brain regions to investigate neural underpinnings of persisting perception of background pain in patients with complex regional pain syndrome (CRPS). A total of 25 patients with CRPS and 25 matched healthy controls underwent functional magnetic resonance imaging at rest. With the anterior and posterior insular cortices as seed regions, we compared the strength of the resting-state functional connectivity between the two groups. Functional connectivity between the anterior and posterior insular cortices and the postcentral and inferior frontal gyri, cingulate cortices was reduced in patients with CRPS compared with controls. Additionally, greater reductions in functional connectivity between the anterior insula and right postcentral gyrus were associated with more severe sensory pain in patients with CRPS (short-form McGill Pain Questionnaire sensory subscores, r = -.517, P = .023). The present results imply a possible role of the insula in aberrant processing of pain information in patients with CRPS. The findings suggest that a functional derangement of the connection between one of the somatosensory cortical functions of perception and one of the insular functions of awareness can play a significant role in the persistent experience of regional pain that is not confined to a specific nerve territory. PMID:28692702

  10. Critical Reflectance Derived from MODIS: Application for the Retrieval of Aerosol Absorption over Desert Regions

    Science.gov (United States)

    Wells, Kelley C.; Martins, J. Vanderlei; Remer, Lorraine A.; Kreidenweis, Sonia M.; Stephens, Graeme L.

    2012-01-01

    Aerosols are tiny suspended particles in the atmosphere that scatter and absorb sunlight. Smoke particles are aerosols, as are sea salt, particulate pollution and airborne dust. When you look down at the earth from space sometimes you can see vast palls of whitish smoke or brownish dust being transported by winds. The reason that you can see these aerosols is because they are reflecting incoming sunlight back to the view in space. The reason for the difference in color between the different types of aerosol is that the particles arc also absorbing sunlight at different wavelengths. Dust appears brownish or reddish because it absorbs light in the blue wavelengths and scatters more reddish light to space, Knowing how much light is scattered versus how much is absorbed, and knowin that as a function of wavelength is essential to being able to quantify the role aerosols play in the energy balance of the earth and in climate change. It is not easy measuring the absorption properties of aerosols when they are suspended in the atmosphere. People have been doing this one substance at a time in the laboratory, but substances mix when they are in the atmosphere and the net absorption effect of all the particles in a column of air is a goal of remote sensing that has not yet been completely successful. In this paper we use a technique based on observing the point at which aerosols change from brightening the surface beneath to darkening it. If aerosols brighten a surface. they must scatter more light to space. If they darken the surface. they must be absorbing more. That cross over point is called the critical reflectance and in this paper we show that critical reflectance is a monotonic function of the intrinsic absorption properties of the particles. This parameter we call the single scattering albedo. We apply the technique to MODIS imagery over the Sahara and Sahel regions to retrieve the single scattering albedo in seven wavelengths, compare these retrievals to ground

  11. Impaired spatial body representation in complex regional pain syndrome type 1 (CRPS I).

    Science.gov (United States)

    Reinersmann, Annika; Landwehrt, Julia; Krumova, Elena K; Ocklenburg, Sebastian; Güntürkün, Onur; Maier, Christoph

    2012-11-01

    Recently, a shift of the visual subjective body midline (vSM), a correlate of the egocentric reference frame, towards the affected side was reported in patients with complex regional pain syndrome (CRPS). However, the specificity of this finding is as yet unclear. This study compares 24 CRPS patients to 21 patients with upper limb pain of other origin (pain control) and to 24 healthy subjects using a comprehensive test battery, including assessment of the vSM in light and dark, line bisection, hand laterality recognition, neglect-like severity symptoms, and motor impairment (disability of the arm, shoulder, and hand). 1-way analysis of variance, t-tests, significance level: 0.05. In the dark, CRPS patients displayed a significantly larger leftward spatial bias when estimating their vSM, compared to pain controls and healthy subjects, and also reported lower motor function than pain controls. For right-affected CRPS patients only, the deviation of the vSM correlated significantly with the severity of distorted body perception. Results confirm previous findings of impaired visuospatial perception in CRPS patients, which might be the result of the involvement of supraspinal mechanisms in this pain syndrome. These mechanisms might accentuate the leftward bias that results from a right-hemispheric dominance in visuospatial processing and is known as pseudoneglect. Pseudoneglect reveals itself in the tendency to perceive the midpoint of horizontal lines or the subjective body midline left of the centre. It was observable in all 3 groups, but most pronounced in CRPS patients, which might be due to the cortical reorganisation processes associated with this syndrome. Copyright © 2012 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  12. Prevalence and characteristics of fetal alcohol syndrome and partial fetal alcohol syndrome in a Rocky Mountain Region City.

    Science.gov (United States)

    May, Philip A; Keaster, Carol; Bozeman, Rosemary; Goodover, Joelene; Blankenship, Jason; Kalberg, Wendy O; Buckley, David; Brooks, Marita; Hasken, Julie; Gossage, J Phillip; Robinson, Luther K; Manning, Melanie; Hoyme, H Eugene

    2015-10-01

    The prevalence and characteristics of fetal alcohol syndrome (FAS) and partial FAS (PFAS) in the United States (US) are not well known. This active case ascertainment study in a Rocky Mountain Region City assessed the prevalence and traits of children with FAS and PFAS and linked them to maternal risk factors. Diagnoses made by expert clinical dysmorphologists in multidisciplinary case conferences utilized all components of the study: dysmorphology and physical growth, neurobehavior, and maternal risk interviews. Direct parental (active) consent was obtained for 1278 children. Averages for key physical diagnostic traits and several other minor anomalies were significantly different among FAS, PFAS, and randomly-selected, normal controls. Cognitive tests and behavioral checklists discriminated the diagnostic groups from controls on 12 of 14 instruments. Mothers of children with FAS and PFAS were significantly lower in educational attainment, shorter, later in pregnancy recognition, and suffered more depression, and used marijuana and methamphetamine during their pregnancy. Most pre-pregnancy and pregnancy drinking measures were worse for mothers of FAS and PFAS. Excluding a significant difference in simply admitting drinking during the index pregnancy (FAS and PFAS=75% vs. 39.4% for controls), most quantitative intergroup differences merely approached significance. This community's prevalence of FAS is 2.9-7.5 per 1000, PFAS is 7.9-17.7 per 1000, and combined prevalence is 10.9-25.2 per 1000 or 1.1-2.5%. Comprehensive, active case ascertainment methods produced rates of FAS and PFAS higher than predicted by long-standing, popular estimates. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  13. Psychotropic treatments in Prader-Willi syndrome: a critical review of published literature.

    Science.gov (United States)

    Bonnot, O; Cohen, D; Thuilleaux, D; Consoli, A; Cabal, S; Tauber, M

    2016-01-01

    Prader-Willi syndrome (PWS) is a rare genetic syndrome. The phenotype includes moderate to intellectual disability, dysmorphia, obesity, and behavioral disturbances (e.g., hetero and self-injurious behaviors, hyperphagia, psychosis). Psychotropic medications are widely prescribed in PWS for symptomatic control. We conducted a systematic review of published literature to examine psychotropic medications used in PWS. MEDLINE was searched to identify articles published between January 1967 and December 2014 using key words related to pharmacological treatments and PWS. Articles with original data were included based on a standardized four-step selection process. The identification of studies led to 241 records. All selected articles were evaluated for case descriptions (PWS and behavioral signs) and treatment (type, titration, efficiency, and side effects). Overall, 102 patients were included in these studies. Treatment involved risperidone (three reports, n = 11 patients), fluoxetine (five/n = 6), naltrexone (two/n = 2), topiramate (two/n = 16), fluvoxamine (one/n = 1), mazindol (one/n = 2), N-acetyl cysteine (one/n = 35), rimonabant (one/n = 15), and fenfluramine (one/n = 15). We identified promising treatment effects with topiramate for self-injury and impulsive/aggressive behaviors, risperidone for psychotic symptoms associated with uniparental disomy (UPD), and N-acetyl cysteine for skin picking. The pharmacological approach of behavioral impairment in PWS has been poorly investigated to date. Further randomized controlled studies are warranted. Behavioral disturbances in Prader-Willi syndrome including aggressive reactions, skin picking, and hyperphagia might be very difficult to manage. Antipsychotic drugs are widely prescribed, but weight gain and increased appetite are their major side effects. Topiramate might be efficient for self-injury and impulsive/aggressive behaviors, N-acetyl cysteine is apromising treatment for

  14. Use of sequential diagnostic pain blocks in a patient of posttraumatic complex regional pain syndrome-not otherwise specified complicated by myofascial trigger points and thoracolumbar pain syndrome

    Directory of Open Access Journals (Sweden)

    Kailash Kothari

    2017-01-01

    Full Text Available We are presenting a case of posttraumatic lower limb Complex regional pain syndrome – Not otherwise specified (CRPS – NOS. As it was not treated in acute phase, the pain became chronic and got complicated by myofascial and thoracolumbar pain syndrome. This case posed us a diagnostic challenge. We used sequential diagnostic pain blocks to identify the pain generators and successfully treat the patient. We used diagnostic blocks step by step to identify and treat pain generators – T12,L1 and L2 Facet joints, Lumbar sympathetic block for CRPS NOS and Trigger point injection with dry needling for myofascial pain syndrome. This case highlights the facet that additional pain generators unrelated to original pain may complicate the presentation. Identifying these pain generators requires out of box thinking and high index of suspicion.

  15. Perceived barriers to the regionalization of adult critical care in the United States: a qualitative preliminary study

    Directory of Open Access Journals (Sweden)

    Rubenfeld Gordon D

    2008-11-01

    Full Text Available Abstract Background Regionalization of adult critical care services may improve outcomes for critically ill patients. We sought to develop a framework for understanding clinician attitudes toward regionalization and potential barriers to developing a tiered, regionalized system of care in the United States. Methods We performed a qualitative study using semi-structured interviews of critical care stakeholders in the United States, including physicians, nurses and hospital administrators. Stakeholders were identified from a stratified-random sample of United States general medical and surgical hospitals. Key barriers and potential solutions were identified by performing content analysis of the interview transcriptions. Results We interviewed 30 stakeholders from 24 different hospitals, representing a broad range of hospital locations and sizes. Key barriers to regionalization included personal and economic strain on families, loss of autonomy on the part of referring physicians and hospitals, loss of revenue on the part of referring physicians and hospitals, the potential to worsen outcomes at small hospitals by limiting services, and the potential to overwhelm large hospitals. Improving communication between destination and source hospitals, provider education, instituting voluntary objective criteria to become a designated referral center, and mechanisms to feed back patients and revenue to source hospitals were identified as potential solutions to some of these barriers. Conclusion Regionalization efforts will be met with significant conceptual and structural barriers. These data provide a foundation for future research and can be used to inform policy decisions regarding the design and implementation of a regionalized system of critical care.

  16. Investigation of regional cerebral blood flow in alcoholic Korsakoff`s syndrome with {sup 123}I-IMP SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Makoto; Nomura, Soichiro [Tachikawa Hospital, Tokyo (Japan); Kato, Motoichiro; Nakazawa, Tsuneyuki

    1995-05-01

    Regional cerebral blood flow (rCBF) was determined quantitatively by single photon emission computed tomography (SPECT) using N-isopropyl-p-[{sup 123}I]iodoamphetamine ({sup 123}I-IMP) in 6 patients with alcoholic Korsakoff`s syndrome (A group). The findings were compared with concurrently available findings from 6 alcoholic patients with no evidence of cerebral disordres such as Korsakoff`s syndrome and dementia (B group) and 4 healthy persons (C group). In both A and B groups, diffuse decrease in blood flow was significantly observed in the entire brain, as compared with the C group; no significant difference in the decreased blood flow existed between the A and B groups. According to the WAIS results, the patients in the A group were classified as `typical Korsakoff`s syndrome` (full IQ of 90 or more) and as `serious Korsakoff`s syndrome` (full IQ of 89 or less). rCBF in the thalamus was significantly lower in the A group of patients with typical Korsakoff`s syndrome than the B group. These findings may reflect the variety of alcoholic Korsakoff`s syndrome. This may also have an implication for the possible classification of several types in this syndrome. (N.K.).

  17. [Social skills in emergency and critical care professionals of a regional public hospital].

    Science.gov (United States)

    Leal Costa, C; Luján Cebrián, I; Gascón García, J; Ferrer Villalonga, L; Van-der Hofstadt Román, C J

    2010-01-01

    To assess the social skills of health care professionals in the emergency and critical care units of the regional hospital Vega Baja and analyze the association between a series of sociodemographic and professionals variables and social skills. A cross-sectional, descriptive study. Two evaluation tools were used. These included a sociodemographic and professional variables questionnaire, and the Elena Gismero's Social Skills Scale (SSS). A response rate of 82.6% was obtained. Considering the standards made by the author in SSS validation, it can be seen that the sample has obtained a medium-high score in each one of the aspects. Significant differences have been found when considering the sociodemographic variable gender as an independent variable with the complete score of SSS (F=6.555; p=0.013), and with the scale dimensions, self-expression in social situations (F=4.468; p=0.039) and making a demand (F=7.982; p=0.007). In each one of the SSS dimensions, the sample has obtained a slightly higher score than the standard sample and it within the 50-69 percentile. This indicates the existence of a medium-high level of Social Skill. The doctors score higher than the nurses, although these differences are not statistically significant. Copyright © 2010 Elsevier España, S.L. y SEEIUC. All rights reserved.

  18. Regions of existence of two forms of the critical void fraction dependence on heat flux density at burnout

    International Nuclear Information System (INIS)

    Smolin, V.N.

    1981-01-01

    On the basis of the available experimental data considered is the burnout during the movement of steam-water flow in vertical heated tubes with internal diameter from 8 to 40 mm. Critical steam content Xsub(cr) dependences on the critical heat flux qsub(cr) in different tubes and under different pressure are analyzed. Two main regions of the weak and strong dependences Xsub(cr)=f(qsub(cr)) at burnout are found out [ru

  19. Association of Neglect-Like Symptoms with Anxiety, Somatization, and Depersonalization in Complex Regional Pain Syndrome.

    Science.gov (United States)

    Michal, Matthias; Adler, Julia; Reiner, Iris; Wermke, Andreas; Ackermann, Tatiana; Schlereth, Tanja; Birklein, Frank

    2017-04-01

    Many patients with complex regional pain syndrome (CRPS) report some foreignness of the affected limb, which is referred to as "neglect-like symptoms" (NLS). Despite similarities of the NLS reports to symptoms of body image disturbances in mental disorders, no study has been conducted to examine such associations. We investigated 50 patients with CRPS and 45 pain control patients (N = 27, chronic limb pain; N = 18, migraine headache). NLS, anxiety, depression, depersonalization, and somatization were assessed using validated questionnaires. Seventy-two percent of the CRPS patients reported at least one NLS vs 29.6% and 33.3% in the two patient control groups. In limb pain controls, NLS correlated with pain intensity. In CRPS patients, NLS correlated with anxiety (rho = 0.658, P  psychological studies. © 2016 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  20. Complex regional pain syndrome with associated chest wall dystonia: a case report

    Directory of Open Access Journals (Sweden)

    Schwartzman Robert J

    2011-09-01

    Full Text Available Abstract Patients with complex regional pain syndrome (CRPS often suffer from an array of associated movement disorders, including dystonia of an affected limb. We present a case of a patient with long standing CRPS after a brachial plexus injury, who after displaying several features of the movement disorder previously, developed painful dystonia of chest wall musculature. Detailed neurologic examination found palpable sustained contractions of the pectoral and intercostal muscles in addition to surface allodynia. Needle electromyography of the intercostal and paraspinal muscles supported the diagnosis of dystonia. In addition, pulmonary function testing showed both restrictive and obstructive features in the absence of a clear cardiopulmonary etiology. Treatment was initiated with intrathecal baclofen and the patient had symptomatic relief and improvement of dystonia. This case illustrates a novel form of the movement disorder associated with CRPS with response to intrathecal baclofen treatment.

  1. Molecular signature of complex regional pain syndrome (CRPS) and its analysis.

    Science.gov (United States)

    König, Simone; Schlereth, Tanja; Birklein, Frank

    2017-10-01

    Complex Regional Pain Syndrome (CRPS) is a rare, but often disabling pain disease. Biomarkers are lacking, but several inflammatory substances have been associated with the pathophysiology. This review outlines the current knowledge with respect to target biomolecules and the analytical tools available to measure them. Areas covered: Targets include cytokines, neuropeptides and resolvins; analysis strategies are thus needed for different classes of substances such as proteins, peptides, lipids and small molecules. Traditional methods like immunoassays are of importance next to state-of-the art high-resolution mass spectrometry techniques and 'omics' approaches. Expert commentary: Future biomarker studies need larger cohorts, which improve subgrouping of patients due to their presumed pathophysiology, and highly standardized workflows from sampling to analysis.

  2. Continuous Thoracic Sympathetic Ganglion Block in Complex Regional Pain Syndrome Patients with Spinal Cord Stimulation Implantation

    Directory of Open Access Journals (Sweden)

    EungDon Kim

    2016-01-01

    Full Text Available The sympathetic block is widely used for treating neuropathic pain such as complex regional pain syndrome (CRPS. However, single sympathetic block often provides only short-term effect. Moreover, frequent procedures for sympathetic block may increase the risk of complications. The use of epidural route may be limited by concern of infection in case of previous implantation of the spinal cord stimulation (SCS. In contrast, a continuous sympathetic block can be administered without such concerns. The continuous thoracic sympathetic block (TSGB has been used to treat the ischemic disease and other neuropathic conditions such as postherpetic neuralgia. We administered continuous thoracic sympathetic block using catheter in CRPS patients who underwent SCS implantations and achieved desirable outcomes. We believe a continuous sympathetic block is a considerable option before performing neurolysis or radiofrequency rhizotomy and even after SCS implantation.

  3. Measurements of saturation densities in critical region and critical loci for binary R-32/125 and R-125/143a systems

    Energy Technology Data Exchange (ETDEWEB)

    Kishizawa, G.; Sato, H.; Watanabe, K.

    1999-05-01

    R-32/125 (difluoromethane/pentafluoroethane) and R-125/143a (pentafluoroethane/1,1,1-trifluoroethane) binary systems are promising alternative refrigerants to replace conventional refrigerants, i.e., R-22 and R-502. The saturated vapor- and liquid-density data in the critical region of these mixtures were measured using the visual observation of the miniscus disappearance in an optical cell. For the R-32/125 system, 35 saturation density data were measured at three compositions, 10, 35, and 50 mass % R-32. Nineteen saturation density data were also measured for R-125/143a (50/50 mass %). The critical temperatures and densities for these binary refrigerants were determined by taking into consideration the level and location of the meniscus disappearance as well as the intensity of the critical opalescence. Correlations to represent the critical loci of these binary refrigerants for an entire range of compositions have been developed. The experimental uncertainties of the saturation density data are estimated to be within 9 mK in temperature and 0.5 to 5.0 kg{center{underscore}dot}m{sup {minus}3} in density. The uncertainties of the critical temperature and density are estimated to be within 12 to 14 mK and 4 to 8 kg{center{underscore}dot}m{sup {minus}3}, respectively.

  4. Venipuncture-Induced Complex Regional Pain Syndrome: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Foad Elahi

    2014-01-01

    Full Text Available Venipuncture, the most frequently performed invasive medical procedure, is usually benign. Generally it produces only transitory mild discomfort. Venipuncture-induced neuropathic pain is hard to recognize at an early stage. Medical literature reviews show that there is not adequate medical knowledge about this important subject. The inciting incident in complex regional pain syndrome (CRPS can often seem far too trivial to result in a condition with such severe pathophysiologic effects. The practicing physician has little information available to enable early recognition of the condition, initiation of multidisciplinary treatment modalities, and proper referral to pain specialists. We encountered a unique case of venipuncture-induced complex regional pain syndrome (CRPS. The patient is a 52-year-old school teacher with no significant past medical history, who presented initially to the Center of Pain Medicine with left upper extremity pain. The pain started while phlebotomy was performed in the patient’s left antecubital area for routine blood check. The patient’s pain did not improve with multiple medications, physical therapy, or several nerve blocks. The patient demonstrated all the signs and symptoms of chronic neuropathic pain of CRPS in the upper extremity with minimal response to the continuous pain management. We decided to proceed with cervical spinal cord nerve stimulation along with continuing other modalities. The patient responded to this combination. During the follow-up, we noticed that the patient’s pain course was complicated by extension of the CRPS to her lower extremity. We will describe the course of treatment for the patient in this paper. In this paper we will discuss the electrical neuromodulation as an important modality in addition to the multidisciplinary pain management for a patient with venipuncture-induced chronic neuropathic pain.

  5. Lack of genetic association of neutral endopeptidase (NEP) with complex regional pain syndrome (CRPS).

    Science.gov (United States)

    Huehne, Kathrin; Schaal, Ute; Leis, Stefan; Uebe, Steffen; Gosso, M Florencia; van den Maagdenberg, Arn M J M; Maihöfner, Christian; Birklein, Frank; Rautenstrauss, Bernd; Winterpacht, Andreas

    2010-03-12

    Complex regional pain syndrome (CRPS) is a condition that is characterized by severe pain and exaggerated neurogenic inflammation, which may develop after injury or surgery. Neurogenic inflammation is mediated by neuropeptides, such as calcitonin gene-related peptide (CGRP) and substance P (SP) that are released from nociceptors. Genetic factors may play a role in CRPS as was suggested by the occurrence of familial cases and several genetic association studies investigating mainly the human leukocyte antigen (HLA) system. Here we investigated the role of neutral endopeptidase (NEP), a key enzyme in neuropeptide catabolism. NEP dysfunction resulting in reduced inactivation of neuropeptides may be a possible pathomechanism in CRPS. To this end, we tested a GT-repeat polymorphism in the NEP promoter region as well as 18 tag-SNPs in six linkage disequilibrium (LD) blocks in the NEP gene region in 320 CRPS patients and 376 controls. No significant genetic association was observed. Thus, we conclude that the NEP gene does not seem to be a major risk factor for CRPS. Copyright 2010 Elsevier Ireland Ltd. All rights reserved.

  6. Low regional cerebral blood flow in burning mouth syndrome patients with depression.

    Science.gov (United States)

    Liu, B-L; Yao, H; Zheng, X-J; Du, G-H; Shen, X-M; Zhou, Y-M; Tang, G-Y

    2015-07-01

    The main aims of this study were to (i) investigate the emotional disorder status of patients with burning mouth syndrome (BMS) and (ii) detect regional cerebral blood flow in BMS patients with the application of combined single-photon emission computed tomography and computed tomography (SPECT/CT). The degree of pain was measured using the visual analysis scale, and emotional disorder with the self-rating anxiety scale, self-rating depression scale, and Hamilton depression rating scale in 29 patients with BMS and 10 healthy controls. SPECT/CT was performed in 29 patients with BMS and 10 healthy controls, and statistical parametric mapping method was used for between-group analyses. The incidence rate of depression in patients with BMS was 31.0%. Compared to the control group, patients with BMS displayed significantly different depression and anxiety scales (P < 0.05). Significantly lower regional cerebral blood flow in the left parietal and left temporal lobes was recorded for BMS patients with depression (P < 0.05). Patients with BMS experience more depression and anxious emotion. Moreover, depression in patients with BMS may be associated with lower regional cerebral blood flow in the left temporal and left parietal lobes. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Regional cerebral glucose metabolism is normal in young adults with Down syndrome

    International Nuclear Information System (INIS)

    Schapiro, M.B.; Grady, C.L.; Kumar, A.; Herscovitch, P.; Haxby, J.V.; Moore, A.M.; White, B.; Friedland, R.P.; Rapoport, S.I.

    1990-01-01

    Regional CMRglc (rCMRglc) values were measured with [ 18 F]2-fluoro-2-deoxy-D-glucose ( 18 FDG) and positron emission tomography (PET), using a Scanditronix PC-1024-7B scanner, in 14 healthy, noninstitutionalized subjects with trisomy 21 (Down syndrome; DS) (mean age 30.0 years, range 25-38 years) and in 13 sex-matched, healthy volunteers (mean age 29.5 years, range 22-38 years). In the DS group, mean mental age on the Peabody Picture Vocabulary Test was 7.8 years and dementia was not present. Resting rCMRglc was determined with eyes covered and ears occluded in a quiet, darkened room. Global gray CMRglc equaled 8.76 +/- 0.76 mg/100 g/min (mean +/- SD) in the DS group as compared with 8.74 +/- 1.19 mg/100 g/min in the control group (p greater than 0.05). Gray matter regional measurements also did not differ between groups. The ratio of rCMRglc to global CMRglc, calculated to reduce the variance associated with absolute rCMRglc, and right/left ratios did not show any consistent differences. These results show that healthy young DS adults do not have alterations in regional or global brain glucose metabolism, as measured with 18FDG and PET, prior to an age at which the neuropathological changes in Alzheimer disease are reported to occur

  8. The liquid–liquid coexistence curves of {benzonitrile + n-pentadecane} and {benzonitrile + n-heptadecane} in the critical region

    International Nuclear Information System (INIS)

    Chen, Zhiyun; Bai, Yongliang; Yin, Tianxiang; An, Xueqin; Shen, Weiguo

    2012-01-01

    Highlights: ► Coexistence curves of (benzonitrile + n-pentadecane) and (benzonitrile + n-heptadecane) were measured. ► The values of the critical exponent β are consistent with that predicted by the 3D-Ising model. ► The coexistence curves are well described by the critical crossover model. ► The asymmetry of the diameters of the coexistence curves were discussed by the complete scaling theory. - Abstract: Liquid + liquid coexistence curves for the binary solutions of {benzonitrile + n-pentadecane} and {benzonitrile + n-heptadecane} have been measured in the critical region. The critical exponent β and the critical amplitudes have been deduced and the former is consistent with the theoretic prediction. It was found that the coexistence curves may be well described by the crossover model proposed by Gutkowski et al. The asymmetries of the diameters of the coexistence curves were also discussed in the frame of the complete scaling theory.

  9. Measuring Progress on the Control of Porcine Reproductive and Respiratory Syndrome (PRRS at a Regional Level: The Minnesota N212 Regional Control Project (Rcp as a Working Example.

    Directory of Open Access Journals (Sweden)

    Pablo Valdes-Donoso

    Full Text Available Due to the highly transmissible nature of porcine reproductive and respiratory syndrome (PRRS, implementation of regional programs to control the disease may be critical. Because PRRS is not reported in the US, numerous voluntary regional control projects (RCPs have been established. However, the effect of RCPs on PRRS control has not been assessed yet. This study aims to quantify the extent to which RCPs contribute to PRRS control by proposing a methodological framework to evaluate the progress of RCPs. Information collected between July 2012 and June 2015 from the Minnesota Voluntary Regional PRRS Elimination Project (RCP-N212 was used. Demography of premises (e.g. composition of farms with sows = SS and without sows = NSS was assessed by a repeated analysis of variance. By using general linear mixed-effects models, active participation of farms enrolled in the RCP-N212, defined as the decision to share (or not to share PRRS status, was evaluated and used as a predictor, along with other variables, to assess the PRRS trend over time. Additionally, spatial and temporal patterns of farmers' participation and the disease dynamics were investigated. The number of farms enrolled in RCP-N212 and its geographical coverage increased, but the proportion of SS and NSS did not vary significantly over time. A significant increasing (p<0.001 trend in farmers' decision to share PRRS status was observed, but with NSS producers less willing to report and a large variability between counties. The incidence of PRRS significantly (p<0.001 decreased, showing a negative correlation between degree of participation and occurrence of PRRS (p<0.001 and a positive correlation with farm density at the county level (p = 0.02. Despite a noted decrease in PRRS, significant spatio-temporal patterns of incidence of the disease over 3-weeks and 3-kms during the entire study period were identified. This study established a systematic approach to quantify the effect of RCPs on

  10. Negative Symptom Dimensions of the Positive and Negative Syndrome Scale Across Geographical Regions: Implications for Social, Linguistic, and Cultural Consistency.

    Science.gov (United States)

    Khan, Anzalee; Liharska, Lora; Harvey, Philip D; Atkins, Alexandra; Ulshen, Daniel; Keefe, Richard S E

    2017-12-01

    Objective: Recognizing the discrete dimensions that underlie negative symptoms in schizophrenia and how these dimensions are understood across localities might result in better understanding and treatment of these symptoms. To this end, the objectives of this study were to 1) identify the Positive and Negative Syndrome Scale negative symptom dimensions of expressive deficits and experiential deficits and 2) analyze performance on these dimensions over 15 geographical regions to determine whether the items defining them manifest similar reliability across these regions. Design: Data were obtained for the baseline Positive and Negative Syndrome Scale visits of 6,889 subjects across 15 geographical regions. Using confirmatory factor analysis, we examined whether a two-factor negative symptom structure that is found in schizophrenia (experiential deficits and expressive deficits) would be replicated in our sample, and using differential item functioning, we tested the degree to which specific items from each negative symptom subfactor performed across geographical regions in comparison with the United States. Results: The two-factor negative symptom solution was replicated in this sample. Most geographical regions showed moderate-to-large differential item functioning for Positive and Negative Syndrome Scale expressive deficit items, especially N3 Poor Rapport, as compared with Positive and Negative Syndrome Scale experiential deficit items, showing that these items might be interpreted or scored differently in different regions. Across countries, except for India, the differential item functioning values did not favor raters in the United States. Conclusion: These results suggest that the Positive and Negative Syndrome Scale negative symptom factor can be better represented by a two-factor model than by a single-factor model. Additionally, the results show significant differences in responses to items representing the Positive and Negative Syndrome Scale expressive

  11. Thermodynamic behavior of {ethanol + butylpyridinium tetrafluoroborate} binary solution in the critical region

    International Nuclear Information System (INIS)

    Tao, Xiaoyi; Yin, Tianxiang; Xu, Chen; Shen, Weiguo

    2017-01-01

    Highlights: • Coexistence curve, heat capacity and turbidity measurements were performed. • RTIL solution showed solvophobic criticality. • Universal critical amplitude ratios were testified. • Asymmetric behavior of the diameter of coexistence curve was discussed. - Abstract: The liquid-liquid coexistence curve, heat capacity, and turbidity of binary solution {ethanol + butylpyridinium tetrafluoroborate]} have been precisely measured. The critical exponents and critical amplitudes corresponding to the heat capacity, width of coexistence curve, osmotic compressibility, and correlation length were obtained. The critical exponents and critical amplitude ratios showed well agreements with the theoretical values of the 3D-Ising universality class. The asymmetric behavior of the coexistence curve diameter was found to be well described by the complete scaling theory with the consideration of the heat capacity related term.

  12. [Lateral hostilities among emergency and critical care nurses. Survey in five hospitals of the Tuscany Region].

    Science.gov (United States)

    Bambi, Stefano; Becattini, Giovanni; Pronti, Fabio; Lumini, Enrico; Rasero, Laura

    2013-01-01

    Lateral hostilities among emergency and critical care nurses. Survey in five hospitals of Tuscany Region. Introduction. Lateral hostilities (LHs) are a kind of workplace violence. They are defined as varieties of cruel, rude, antagonistic interactions between people at the same hierarchical level. Nurses are affected by LH from 5.7% to 65%, leading to reduced work motivation, psycho-physical disorders, and in some cases, drop out of the nursing profession. Objective. To quantify the LHs among nurses in the emergency departments (ED) and intensive care units (ICU) in 5 hospitals of Tuscany (Italy). To show the impact on the quality of their psycho-physical and professional lives. Method. Exploratory-descriptive study, through closed-ended questionnaire. Results. 360/444 nurses (81%); 294 (81.6%) were victims of LHs during the past 12 months. Gossiping, complaints shared with others without discussing with the concerned person, and sarcastic comments were the most reported LHs. LHs occur more in EDs than ICUs (respectively 90% and 77%; p=0.0038). No statistically significant differences were observed for gender, age, or years of experience. The 17.7% of nurses asked to be moved from the ward, and 6.9% left it; 6.9% respondents had thought to leave the nursing profession; 235 (65.2%) experienced at least one LHs related disorder during the last year. Most reported symptoms were low morale, anxiety, and sleep disturbances. Conclusions. The incidence of LH and related disorders is high in EDs and ICUs, determining a low professional and psycho-physical quality of life.

  13. Motor dysfunction in complex regional pain syndrome : the role of sensory processing and sensory-motor integration

    NARCIS (Netherlands)

    Bank, Paulina Johanna Maria

    2014-01-01

    In the chronic stage of Complex Regional Pain Syndrome (CRPS), motor disturbances are common and cause significant disability. The motor dysfunction of CRPS is a poorly understood phenomenon that is characterized predominantly by a decrease or loss of voluntary muscle control. This thesis aims to

  14. fMRI Reveals Distinct CNS Processing during Symptomatic and Recovered Complex Regional Pain Syndrome in Children

    Science.gov (United States)

    Lebel, A.; Becerra, L.; Wallin, D.; Moulton, E. A.; Morris, S.; Pendse, G.; Jasciewicz, J.; Stein, M.; Aiello-Lammens, M.; Grant, E.; Berde, C.; Borsook, D.

    2008-01-01

    Complex regional pain syndrome (CRPS) in paediatric patients is clinically distinct from the adult condition in which there is often complete resolution of its signs and symptoms within several months to a few years. The ability to compare the symptomatic and asymptomatic condition in the same individuals makes this population interesting for the…

  15. Investigating Reports of Complex Regional Pain Syndrome: An Analysis of HPV-16/18-Adjuvanted Vaccine Post-Licensure Data

    NARCIS (Netherlands)

    F.J.P.M. Huygen (Frank); K. Verschueren (Kristin); C. McCabe (Candida); J.-U. Stegmann (Jens-Ulrich); J. Zima (Julia); O. Mahaux (Olivia); L. Van Holle (Lionel); M.-G. Angelo (Maria-Genalin)

    2015-01-01

    textabstractComplex regional pain syndrome (CRPS) is a chronic pain disorder that typically follows trauma or surgery. Suspected CRPS reported after vaccination with human papillomavirus (HPV) vaccines led to temporary suspension of proactive recommendation of HPV vaccination in Japan. We

  16. Impact of spinal cord stimulation on sensory characteristics in complex regional pain syndrome type I - A randomized trial

    NARCIS (Netherlands)

    Kemler, MA; Reulen, JPH; Barendse, GAM; van Kleef, M; de Vet, HCW; van den Wildenberg, FAJM

    Background: A randomized trial was performed to assess the effect of spinal cord stimulation (SCS) on detection and pain thresholds for pressure, warmth, and cold and on the extent of mechanical hyperalgesia in patients with chronic complex regional pain syndrome type I. Methods: Fifty-four chronic

  17. Moral distress and its contribution to the development of burnout syndrome among critical care providers.

    Science.gov (United States)

    Fumis, Renata Rego Lins; Junqueira Amarante, Gustavo Adolpho; de Fátima Nascimento, Andréia; Vieira Junior, José Mauro

    2017-12-01

    Burnout appears to be common among critical care providers. It is characterized by three components: emotional exhaustion, depersonalization and personal accomplishment. Moral distress is the inability of a moral agent to act according to his or her core values and perceived obligations due to internal and external constraints. We aimed to estimate the correlation between moral distress and burnout among all intensive care unit (ICU) and the step-down unit (SDU) providers (physicians, nurses, nurse technicians and respiratory therapists). A survey was conducted from August to September 2015. For data collection, a self-administered questionnaire for each critical care provider was used including basic demographic data, the Maslach Burnout Inventory (MBI) and the Moral Distress Scale-Revised (MDS-R). Correlation analysis between MBI domains and moral distress score and regression analysis to assess independent variables associated with burnout were performed. A total of 283 out of 389 (72.7%) critical care providers agreed to participate. The same team of physicians attended both ICU and SDU, and severe burnout was identified in 18.2% of them. Considering all others critical care providers of both units, we identified that overall 23.1% (95% CI 18.0-28.8%) presented severe burnout, and it did not differ between professional categories. The mean MDS-R rate for all ICU and SDU respondents was 111.5 and 104.5, respectively, p = 0.446. Many questions from MDS-R questionnaire were significantly associated with burnout, and those respondents with high MDS-R score (>100 points) were more likely to suffer from burnout (28.9 vs 14.4%, p = 0.010). After regression analysis, moral distress was independently associated with burnout (OR 2.4, CI 1.19-4.82, p = 0.014). Moral distress, resulting from therapeutic obstinacy and the provision of futile care, is an important issue among critical care providers' team, and it was significantly associated with severe burnout.

  18. Mirror box therapy added to cognitive behavioural therapy in three chronic complex regional pain syndrome type I patients : a pilot study

    NARCIS (Netherlands)

    Tichelaar, Y. I. G. Vladimir; Geertzen, Jan H. B.; Keizer, Doeke; van Wilgen, C. Paul

    Complex regional pain syndrome type I is a disorder of the extremities with disability and pain as the most prominent features. This paper describes the results of cognitive behavioural therapy combined with mirror box therapy in three patients with chronic complex regional pain syndrome type I.

  19. The semiquantitative three-phase bone scintigraphy on hemiplegic patients with earlier complex regional pain syndrome

    International Nuclear Information System (INIS)

    Li Fang; Liu Xingdang; Lu Zhihui; Liu Congjin

    2010-01-01

    Objective: To investigate the difference between the early phases and delay phase of three-phase bone scintigraphy on hemiplegic patients with earlier complex regional pain syndrome (CRPS). Methods: Twenty-nine stroke patients with hemiplegia complicating CRPS received three-phase bone scintigraphy after intravenous injection of 99 Tc m -methylene diphosphonate (MDP). The region of interest (ROI) technique was used to obtain the radioactive counts of involved joints and contralateral sites on wrists, metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints. The total counts of these four sites in each patient were then obtained and the total uptake ratios of involved joints/contralateral joints for each phase were calculated to compare the difference among the three phases. Wilcoxon test and ANOVA were used in data analyses. Results: The involved joints of hemiplegic side displayed higher tracer uptake. There were significant differences of the radioactive counts between involved joints and uninvolved ones in the perfusion, pool and delay phase (Wilcoxon test, Z: -4.73 to -2.10, P<0.05). There was no significant difference of total uptake ratios of involved joints/contralateral joints among the three phases (ANOVA, F = 0. 807, P < 0.05). Conclusions: Due to higher bone seeking agent accumulation on three-phase bone scintigraphy, both early phases and delay phase imaging showed similar value in stroke patients with hemiplegia complicating earlier CRPS. (authors)

  20. Ventilation with lower tidal volumes for critically ill patients without the acute respiratory distress syndrome: a systematic translational review and meta-analysis

    NARCIS (Netherlands)

    Serpa Neto, Ary; Nagtzaam, Liselotte; Schultz, Marcus J.

    2014-01-01

    There is convincing evidence for benefit from lung-protective mechanical ventilation with lower tidal volumes in patients with the acute respiratory distress syndrome (ARDS). It is uncertain whether this strategy benefits critically ill patients without ARDS as well. This manuscript systematically

  1. Quantitative fluorescence-polymerase chain reaction assay for the detection of the duplication of the Charcot Marie Tooth disease type 1A critical region.

    Science.gov (United States)

    De Toffol, Simona; Bellone, Emilia; Dulcetti, Francesca; Ruggeri, Anna Maria; Maggio, Pietro Paolo; Pulimeno, Maria Rosaria; Mandich, Paola; Maggi, Federico; Simoni, Giuseppe; Grati, Francesca Romana

    2010-04-01

    Charcot Marie Tooth (CMT) syndrome is the most common hereditary peripheral neuropathy, with an incidence of about 1 in 2500. The subtype 1A (CMT1A) is caused by a tandem duplication of a 1.5-Mb region encompassing the PMP22 gene. Conventional short tandem repeat (STR) analysis can reveal this imbalance if a triallelic pattern, defining with certainty the presence of duplication, is present. In case of duplication with a biallelic pattern, it can only indicate a semiquantitative dosage of the fluorescence intensity ratio of the two fragments. In this study we developed a quantitative fluorescence-PCR using seven highly informative STRs within the CMT1A critical region that successfully disclosed or excluded the presence of the pathogenic imbalance in a cohort of 60 samples including 40 DNAs from samples with the CMT1A duplication previously characterized with two different molecular approaches, and 20 diagnostic samples from 10 members of a five-generation pedigree segregating CMT1A, 8 unrelated cases and 2 prenatal samples. The application of the quantitative fluorescence-PCR using STRs located in the critical region could be a reliable method to evaluate the presence of the PMP22 duplication for the diagnosis and classification of hereditary neuropathies in asymptomatic subjects with a family history of inherited neuropathy, in prenatal samples in cases with one affected parent, and in unrelated patients with a sporadic demyelinating neuropathy with clinical features resembling CMT (i.e., pes cavus with hammer toes) or with conduction velocities in the range of CMT1A.

  2. Zitkala-Sa and the Problem of Regionalism: Nations, Narratives, and Critical Traditions

    Science.gov (United States)

    Totten, Gary

    2005-01-01

    Although Yankton Sioux writer Zitkala-Sa (Gertrude Bonnin, 1876-1938) was, as P. Jane Hafen notes, "virtually unknown for many decades," much critical work has appeared since Dexter Fisher's 1979 article,"Zitkala-Sa: Evolution of a Writer." Some critics desiring to bring Zitkala-Sa into the conversation about turn-of-the-century American women…

  3. Critical frequency and maximum electron density of F2 region over four stations in the North American sector

    Czech Academy of Sciences Publication Activity Database

    Ezquer, R. G.; Cabrera, M. A.; López, J. L.; Albornoz, M. R.; Mosert, M.; Marcó, P.; Burešová, Dalia

    2011-01-01

    Roč. 73, č. 4 (2011), s. 420-429 ISSN 1364-6826 Institutional research plan: CEZ:AV0Z30420517 Keywords : Ionosphere * F2 region * Critical frequency * Electron density * Model Subject RIV: DG - Athmosphere Sciences, Meteorology Impact factor: 1.596, year: 2011 http://www.sciencedirect.com/science/article/pii/S1364682610002786

  4. Identifying Hot Spots of Critical Forage Supply in Dryland Nomadic Pastoralist Areas: A Case Study for the Afar Region, Ethiopia

    NARCIS (Netherlands)

    Sonneveld, B.G.J.S.; Keyzer, M.A.; van Wesenbeeck, C.F.A.; Georgis, Kidane; Beyene, Fekadu; Urbano, Ferdinando; Meroni, Michele; Leo, Olivier; Yimer, Merkebu; Abdullatif, Mohammed

    2017-01-01

    This study develops a methodology to identify hot spots of critical forage supply in nomadic pastoralist areas, using the Afar Region, Ethiopia, as a special case. It addresses two main problems. First, it makes a spatially explicit assessment of fodder supply and demand extracted from a data poor

  5. Critical regions with central charge c=1/2,7/10,4/5 in the spin-1 quantum chain

    International Nuclear Information System (INIS)

    Mueller, E.

    1991-01-01

    The phase diagramm of the Blume-Emery-Griffiths spin-1-quantum chain is calculated by finite-size scaling with respect to all four parameters. We locate the three-dimensional critical manifold and determine a two-dimensional tricritical surface where the spectra exhibit conformal invariance corresponding to the central charges c=7/10 and 4/5. Choosing one parameter to be zero, we can treat the model analytically and from this the spectrum on a large part of the Ising-like critical region can be understood: there the spectrum consists of conformal c=1/2-levels on which a massive spectrum is superimposed. Calculating three-point functions we study which perturbations by primary fields lead from c=4/5 or c=7/10-critical points to Ising-type regions. (orig.) [de

  6. Short bowel syndrome in infants: the critical role of luminal nutrients in a management program.

    Science.gov (United States)

    Roy, Claude C; Groleau, Véronique; Bouthillier, Lise; Pineault, Marjolain; Thibault, Maxime; Marchand, Valérie

    2014-07-01

    Short bowel syndrome develops when the remnant mass of functioning enterocytes following massive resections cannot support growth or maintain fluid-electrolyte balance and requires parenteral nutrition. Resection itself stimulates the intestine's inherent ability to adapt morphologically and functionally. The capacity to change is very much related to the high turnover rate of enterocytes and is mediated by several signals; these signals are mediated in large part by enteral nutrition. Early initiation of enteral feeding, close clinical monitoring, and ongoing assessment of intestinal adaptation are key to the prevention of irreversible intestinal failure. The length of the functional small bowel remnant is the most important variable affecting outcome. The major objective of intestinal rehabilitation programs is to achieve early oral nutritional autonomy while maintaining normal growth and nutrition status and minimizing total parenteral nutrition related comorbidities such as chronic progressive liver disease. Remarkable progress has been made in terms of survivability and quality of life, especially in the context of coordinated multidisciplinary programs, but much work remains to be done.

  7. Are Shorter Versions of the Positive and Negative Syndrome Scale (PANSS) Doable? A Critical Review.

    Science.gov (United States)

    Lindenmayer, Jean-Pierre

    2017-12-01

    The Positive and Negative Syndrome Scale (PANSS) is a well-established assessment tool for measuring symptom severity in schizophrenia. Researchers and clinicians have been interested in the development of a short version of the PANSS that could reduce the burden of its administration for patients and raters. The author presents a comprehensive overview of existing brief PANSS measures, including their strengths and limitations, and discusses some possible next steps. There are two available scales that offer a reduced number of original PANSS items: PANSS-14 and PANSS-19; and two shorter versions that include six items: Brief PANSS and PANSS-6. The PANSS-6 has been tested quite extensively in established trials and appears to demonstrate high sensitivity to change and an established cut off definition for remission. Prospective testing in new antipsychotic treatment trials is still required for these shorter versions of PANSS. In addition, they need to be supplemented with interview guides, as well as provide conversion formulas to translate total scores from the short PANSS versions to the PANSS-30. Both short versions of the PANSS are essentially designed to evaluate response to antipsychotic treatment. Future PANSS scale development needs to address specific measurement of treatment-responsive positive symptoms by including treatment-sensitive items, as well as illness-phase specific PANSS tools.

  8. A new microduplication syndrome encompassing the region of the Miller-Dieker (17p13 deletion) syndrome

    DEFF Research Database (Denmark)

    Roos, L; Jønch, A E; Kjaergaard, S

    2009-01-01

    BACKGROUND: The use of array comparative genome hybridisation (CGH) analyses for investigation of children with mental retardation has led to the identification of a growing number of new microdeletion and microduplication syndromes, some of which have become clinically well characterised and som...

  9. Determination and Distribution of Critical Loads: Application to the Forest Soils in the Autonomous Region of Madrid

    International Nuclear Information System (INIS)

    Sousa, M.; Schmid, T.; Rabago, I.

    2000-01-01

    The critical loads of acidity and sulphur have been determined for forest soils within the north and northwest of the Autonomous Region of Madrid. The SMB-CCE and SMB-PROFILE steady state models have been applied using a 1 km x 1 km resolution. The forest ecosystems have been characterised according to the soil and forest type, slope and climatic data using a Geographic Information System. In order to estimate the critical loads, processes such as weathering rate of the parent material, atmospheric deposition. critical alkalinity leaching rate and nutrients absorbed by the vegetation have been considered. In general the forest soils present high critical load values for acidity and sulphur. The more sensitive zones are found in the north of the Sierra of Guadarrama. Independent of the applied methods, the results are associated to the types of soils where Leptosols have the lowest, Cambisoles and Regosoles intermediate and Luvisoles the most elevated values. (Author) 40 refs

  10. Tumour MLH1 promoter region methylation testing is an effective prescreen for Lynch Syndrome (HNPCC).

    Science.gov (United States)

    Newton, K; Jorgensen, N M; Wallace, A J; Buchanan, D D; Lalloo, F; McMahon, R F T; Hill, J; Evans, D G

    2014-12-01

    Lynch syndrome (LS) patients have DNA mismatch repair deficiency and up to 80% lifetime risk of colorectal cancer (CRC). Screening of mutation carriers reduces CRC incidence and mortality. Selection for constitutional mutation testing relies on family history (Amsterdam and Bethesda Guidelines) and tumour-derived biomarkers. Initial biomarker analysis uses mismatch repair protein immunohistochemistry and microsatellite instability. Abnormalities in either identify mismatch repair deficiency but do not differentiate sporadic epigenetic defects, due to MLH1 promoter region methylation (13% of CRCs) from LS (4% of CRCs). A diagnostic biomarker capable of making this distinction would be valuable. This study compared two biomarkers in tumours with mismatch repair deficiency; quantification of methylation of the MLH1 promoter region using a novel assay and BRAF c.1799T>A, p.(Val600Glu) mutation status in the identification of constitutional mutations. Tumour DNA was extracted (formalin fixed, paraffin embedded, FFPE tissue) and pyrosequencing used to test for MLH1 promoter methylation and presence of the BRAF c.1799T>A, p.(Val600Glu) mutation 71 CRCs from individuals with pathogenic MLH1 mutations and 73 CRCs with sporadic MLH1 loss. Specificity and sensitivity was compared. Unmethylated MLH1 promoter: sensitivity 94.4% (95% CI 86.2% to 98.4%), specificity 87.7% (95% CI 77.9% to 94.2%), Wild-type BRAF (codon 600): sensitivity 65.8% (95% CI 53.7% to 76.5%), specificity 98.6% (95% CI 92.4% to 100.0%) for the identification of those with pathogenic MLH1 mutations. Quantitative MLH1 promoter region methylation using pyrosequencing is superior to BRAF codon 600 mutation status in identifying constitutional mutations in mismatch repair deficient tumours. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  11. Identification of candidate regions for a novel Usher syndrome type II locus.

    Science.gov (United States)

    Ben Rebeh, Imen; Benzina, Zeineb; Dhouib, Houria; Hadjamor, Imen; Amyere, Mustapha; Ayadi, Leila; Turki, Khalil; Hammami, Bouthaina; Kmiha, Noureddine; Kammoun, Hassen; Hakim, Bochra; Charfedine, Ilhem; Vikkula, Miikka; Ghorbel, Abdelmonem; Ayadi, Hammadi; Masmoudi, Saber

    2008-09-19

    Chronic diseases affecting the inner ear and the retina cause severe impairments to our communication systems. In more than half of the cases, Usher syndrome (USH) is the origin of these double defects. Patients with USH type II (USH2) have retinitis pigmentosa (RP) that develops during puberty, moderate to severe hearing impairment with downsloping pure-tone audiogram, and normal vestibular function. Four loci and three genes are known for USH2. In this study, we proposed to localize the gene responsible for USH2 in a consanguineous family of Tunisian origin. Affected members underwent detailed ocular and audiologic characterization. One Tunisian family with USH2 and 45 healthy controls unrelated to the family were recruited. Two affected and six unaffected family members attended our study. DNA samples of eight family members were genotyped with polymorphic markers. Two-point and multipoint LOD scores were calculated using Genehunter software v2.1. Sequencing was used to investigate candidate genes. Haplotype analysis showed no significant linkage to any known USH gene or locus. A genome-wide screen, using microsatellite markers, was performed, allowing the identification of three homozygous regions in chromosomes 2, 4, and 15. We further confirmed and refined these three regions using microsatellite and single-nucleotide polymorphisms. With recessive mode of inheritance, the highest multipoint LOD score of 1.765 was identified for the candidate regions on chromosomes 4 and 15. The chromosome 15 locus is large (55 Mb), underscoring the limited number of meioses in the consanguineous pedigree. Moreover, the linked, homozygous chromosome 15q alleles, unlike those of the chromosome 2 and 4 loci, are infrequent in the local population. Thus, the data strongly suggest that the novel locus for USH2 is likely to reside on 15q. Our data provide a basis for the localization and the identification of a novel gene implicated in USH2, most likely localized on 15q.

  12. Saturated fat and cholesterol are critical to inducing murine metabolic syndrome with robust nonalcoholic steatohepatitis.

    Science.gov (United States)

    Mells, Jamie E; Fu, Ping P; Kumar, Pradeep; Smith, Tekla; Karpen, Saul J; Anania, Frank A

    2015-03-01

    Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome (MetS). Up to a third of NAFLD subjects are at risk for developing nonalcoholic steatohepatitis (NASH). Many rodent models fail to replicate both MetS and NASH. The purpose of this study was to develop a reliable mouse model of NASH and MetS using a diet containing cholesterol, saturated fat and carbohydrate that is reflective of Western diets of North Americans. We used adult male C57BL/6 J 4- to 5-week-old mice and administered a solid diet containing 0.2% cholesterol, 45% of its calories from fat, with 30% of the fat in the form of partially hydrogenated vegetable oil. We also provided carbohydrate largely as high-fructose corn syrup equivalent in water. In a separate cohort, we gave the identical diet in the absence of cholesterol. Glucose and insulin tolerance testing was conducted throughout the feeding period. The feeding was conducted for 16 weeks, and the mice were sacrificed for histological analysis, markers of MetS, liver inflammation, circulating lipids, as well as liver staining for fibrosis and alpha smooth muscle actin (α-SMA). We found that cholesterol significantly increased serum leptin, interleukin-6, liver weight and liver weight/body weight ratio, fibrosis and liver α-SMA. Mice administered a diet accurately reflecting patterns associated with humans afflicted with MetS can reliably replicate features of MetS, NASH and significant liver fibrosis. The model we describe significantly reduces the time by several months for development of stage 3 hepatic fibrosis. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Global and Regional Left Ventricular Contractile Impairment In Patients With Wolff-Parkinson-White Syndrome

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    Sony Jacob

    2009-07-01

    Full Text Available Background: To assess regional systolic function and global contractile function in patients with WPW Syndrome.Method: Eleven cases with manifest Wolff-Parkinson-White (WPW syndrome in sinus rhythm were compared to 11 age matched controls. 2D strain analysis was performed and peak segmental radial strain (pRS values obtained from basal ventricular parasternal short-axis images (70 ± 5 frames/sec using a dedicated software package. Heterogeneity of radial strain pattern in six circumferential basal left ventricular segments was measured in terms of standard deviations of peak RS (SDpRS or range (difference between maximum and minimum peak RS i.e. RangepRS. Spectral Doppler (continuous wave measurements were acquired through the left ventricular outflow tract to determine Pre Ejection Period (PEP, Left Ventricular Ejection Time (LVET and measures of left ventricular systolic performance. Results: LV segmental radial strain was profoundly heterogeneous in WPW cases in contrast to fairly homogenous strain pattern in normal subjects. Wide SDpRS values 17.5 ± 8.9 vs 3.3 ± 1.4, p<0.001 and RangepRS 42.7 ± 20.8 vs.8.5 ± 3.6 , p<0.001 were observed among WPW and healthy subjects respectively. PEP (132.4 ± 14.7 vs 4.7 ± 0.5ms, p<0.001 and corrected PEP (76.1 ± 8.0 vs 2.7 ± 0.4ms, p<0.001 were significantly longer in WPW patients compared to controls. The PEP/LVET ratio was also significantly greater in WPW cohort (0.49 ± 0.04 vs. 0.28 ± 0.05, p <0.001 suggesting global systolic dysfunction. Conclusion: Patients with manifest preexcitation (predominantly those with right-sided pathways have regional and global contractile dysfunction resulting from aberrant impulse propagation inherent to the preexcited state.

  14. Burnout syndrome in critical care team members: A monocentric cross sectional survey.

    Science.gov (United States)

    Malaquin, Stéphanie; Mahjoub, Yazine; Musi, Arianna; Zogheib, Elie; Salomon, Alexis; Guilbart, Mathieu; Dupont, Hervé

    2017-08-01

    There has been a growing interest in evaluating the occurrence of burnout syndrome (BOS) among intensive care units (ICU) team over recent years. The aims of this study were to determine the prevalence of BOS among staff working in the Amiens University Hospital and to assess associated factors. Prospective observational study based on self-administered questionnaires filled in by physicians and non-physicians working in 3 ICUs. Demographic data, well-being assessment, work relationships, level of BOS and depressive symptoms were investigated. Logistic regression analysis was performed to identify variables independently associated with BOS. One hundred and sixty-one questionnaires were analysed. Participation rate was 90%. Thirty-two respondents were physicians and 129 were non-physicians. The prevalence of BOS was 51% and was not significantly different between physicians and non-physicians (56% versus 50%; P=0.501). Respondents who reported BOS less frequently had regular leisure activities (54 [66%] versus 70 [87%], P=0.001). In the BOS group, well-being was significantly lower (4.8±2.5/10 versus 6±2/10, P=0.001), a desire to leave the job was more frequently expressed (50 [61%] versus 32 [40%], P=0.009) and depressive symptoms were significantly more frequent (41 [50%] versus 21 [27%], P=0.002). Factors independently associated with BOS were regular leisure activities (OR 0.24 [0.1-0.59]; P=0.002), the presence of depressive symptoms (OR 2.71 [1.26-5.84]; P=0.011) and a well-being visual analogue scale≥5 (OR 0.40 [0.18-0.89]; P=0.024). BOS affects all ICU workers and is determined by multiple factors. Leisure activities and measures designed to improve well-being should be promoted. Copyright © 2016 Société française d'anesthésie et de réanimation (Sfar). Published by Elsevier Masson SAS. All rights reserved.

  15. Critical appraisal of canakinumab in the treatment of adults and children with cryopyrin-associated periodic syndrome (CAPS)

    Science.gov (United States)

    Toker, Ori; Hashkes, Philip J

    2010-01-01

    The cryopyrin-associated syndromes (CAPS) include three autosomal-dominant syndromes, that are caused by a mutation in the NLRP3 gene on chromosome 1, encoding the cryopyrin protein. These syndromes, familial cold autoinflammatory syndrome, Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease, are characterized by urticaria-like rash, fever, central nervous system inflammation, an arthropathy and a risk of the development of amyloidosis in a respectively escalating degree of severity between the various syndromes. Recently the role of cryopyrin in the regulation of interleukin (IL)-1 production and activation was described and anti IL-1 therapies were found to be very effective in treating these syndromes. There are several types of anti IL-1 medications based on different mechanisms of antagonizing IL-1. This paper focuses on the efficacy and safety of canakinumab, a long-acting humanized anti IL-1 antibody, in treating these syndromes. PMID:20531965

  16. Utility of Ambulance Data for Real-Time Syndromic Surveillance: A Pilot in the West Midlands Region, United Kingdom.

    Science.gov (United States)

    Todkill, Dan; Loveridge, Paul; Elliot, Alex J; Morbey, Roger A; Edeghere, Obaghe; Rayment-Bishop, Tracy; Rayment-Bishop, Chris; Thornes, John E; Smith, Gillian

    2017-12-01

    Introduction The Public Health England (PHE; United Kingdom) Real-Time Syndromic Surveillance Team (ReSST) currently operates four national syndromic surveillance systems, including an emergency department system. A system based on ambulance data might provide an additional measure of the "severe" end of the clinical disease spectrum. This report describes the findings and lessons learned from the development and preliminary assessment of a pilot syndromic surveillance system using ambulance data from the West Midlands (WM) region in England. Hypothesis/Problem Is an Ambulance Data Syndromic Surveillance System (ADSSS) feasible and of utility in enhancing the existing suite of PHE syndromic surveillance systems? An ADSSS was designed, implemented, and a pilot conducted from September 1, 2015 through March 1, 2016. Surveillance cases were defined as calls to the West Midlands Ambulance Service (WMAS) regarding patients who were assigned any of 11 specified chief presenting complaints (CPCs) during the pilot period. The WMAS collected anonymized data on cases and transferred the dataset daily to ReSST, which contained anonymized information on patients' demographics, partial postcode of patients' location, and CPC. The 11 CPCs covered a broad range of syndromes. The dataset was analyzed descriptively each week to determine trends and key epidemiological characteristics of patients, and an automated statistical algorithm was employed daily to detect higher than expected number of calls. A preliminary assessment was undertaken to assess the feasibility, utility (including quality of key indicators), and timeliness of the system for syndromic surveillance purposes. Lessons learned and challenges were identified and recorded during the design and implementation of the system. The pilot ADSSS collected 207,331 records of individual ambulance calls (daily mean=1,133; range=923-1,350). The ADSSS was found to be timely in detecting seasonal changes in patterns of respiratory

  17. Regional MRI Perfusion Measures Predict Motor/Executive Function in Patients with Clinically Isolated Syndrome

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    Efrosini Z. Papadaki

    2014-01-01

    Full Text Available Background. Patients with clinically isolated syndrome (CIS demonstrate brain hemodynamic changes and also suffer from difficulties in processing speed, memory, and executive functions. Objective. To explore whether brain hemodynamic disturbances in CIS patients correlate with executive functions. Methods. Thirty CIS patients and forty-three healthy subjects, matched for age, gender, education level, and FSIQ, were administered tests of visuomotor learning and set shifting ability. Cerebral blood volume (CBV, cerebral blood flow (CBF, and mean transit time (MTT values were estimated in normal-appearing white matter (NAWM and normal-appearing deep gray Matter (NADGM structures, using a perfusion MRI technique. Results. CIS patients showed significantly elevated reaction time (RT on both tasks, while their CBV and MTT values were globally increased, probably due to inflammatory vasodilation. Significantly, positive correlation coefficients were found between error rates on the inhibition condition of the visuomotor learning task and CBV values in occipital, periventricular NAWM and both thalami. On the set shifting condition of the respective task significant, positive associations were found between error rates and CBV values in the semioval center and periventricular NAWM bilaterally. Conclusion. Impaired executive function in CIS patients correlated positively with elevated regional CBV values thought to reflect inflammatory processes.

  18. Personality assessment of patients with complex regional pain syndrome type I.

    Science.gov (United States)

    Monti, D A; Herring, C L; Schwartzman, R J; Marchese, M

    1998-12-01

    There is controversy regarding the importance of psychological/psychiatric factors in the development of the Complex Regional Pain Syndrome (CRPS). Our objective was to determine whether CRPS type I patients were psychiatrically different from other chronic pain patients, with particular attention to personality pathology. A standardized clinical assessment of all major psychiatric categories, including personality disorders, was performed on 25 CRPS type I patients and a control group of 25 patients with chronic low back pain from disc-related radiculopathy. Both sections of the Structured Clinical Interview for the Diagnostic and Statistical Manual (3rd ed., rev.) and the visual analog scale. Both groups were similar in terms of pain intensity and duration. Statistical analysis showed both groups to have a significant amount of major psychiatric comorbidity, in particular major depressive disorder, and a high incidence of personality disorders. Therefore, intense chronic pain was associated with significant psychiatric comorbidity in both groups and in similar proportions. The high incidence of personality pathology in both groups may represent an exaggeration of maladaptive personality traits and coping styles as a result of a chronic, intense, state of pain.

  19. Proteomic identification of altered cerebral proteins in the complex regional pain syndrome animal model.

    Science.gov (United States)

    Nahm, Francis Sahngun; Park, Zee-Yong; Nahm, Sang-Soep; Kim, Yong Chul; Lee, Pyung Bok

    2014-01-01

    Complex regional pain syndrome (CRPS) is a rare but debilitating pain disorder. Although the exact pathophysiology of CRPS is not fully understood, central and peripheral mechanisms might be involved in the development of this disorder. To reveal the central mechanism of CRPS, we conducted a proteomic analysis of rat cerebrum using the chronic postischemia pain (CPIP) model, a novel experimental model of CRPS. After generating the CPIP animal model, we performed a proteomic analysis of the rat cerebrum using a multidimensional protein identification technology, and screened the proteins differentially expressed between the CPIP and control groups. Results. A total of 155 proteins were differentially expressed between the CPIP and control groups: 125 increased and 30 decreased; expressions of proteins related to cell signaling, synaptic plasticity, regulation of cell proliferation, and cytoskeletal formation were increased in the CPIP group. However, proenkephalin A, cereblon, and neuroserpin were decreased in CPIP group. Altered expression of cerebral proteins in the CPIP model indicates cerebral involvement in the pathogenesis of CRPS. Further study is required to elucidate the roles of these proteins in the development and maintenance of CRPS.

  20. Proteomic Identification of Altered Cerebral Proteins in the Complex Regional Pain Syndrome Animal Model

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    Francis Sahngun Nahm

    2014-01-01

    Full Text Available Background. Complex regional pain syndrome (CRPS is a rare but debilitating pain disorder. Although the exact pathophysiology of CRPS is not fully understood, central and peripheral mechanisms might be involved in the development of this disorder. To reveal the central mechanism of CRPS, we conducted a proteomic analysis of rat cerebrum using the chronic postischemia pain (CPIP model, a novel experimental model of CRPS. Materials and Methods. After generating the CPIP animal model, we performed a proteomic analysis of the rat cerebrum using a multidimensional protein identification technology, and screened the proteins differentially expressed between the CPIP and control groups. Results. A total of 155 proteins were differentially expressed between the CPIP and control groups: 125 increased and 30 decreased; expressions of proteins related to cell signaling, synaptic plasticity, regulation of cell proliferation, and cytoskeletal formation were increased in the CPIP group. However, proenkephalin A, cereblon, and neuroserpin were decreased in CPIP group. Conclusion. Altered expression of cerebral proteins in the CPIP model indicates cerebral involvement in the pathogenesis of CRPS. Further study is required to elucidate the roles of these proteins in the development and maintenance of CRPS.

  1. Abnormal thalamocortical activity in patients with Complex Regional Pain Syndrome (CRPS) type I.

    Science.gov (United States)

    Walton, K D; Dubois, M; Llinás, R R

    2010-07-01

    Complex Regional Pain Syndrome (CRPS) is a neuropathic disease that presents a continuing challenge in terms of pathophysiology, diagnosis, and treatment. Recent studies of neuropathic pain, in both animals and patients, have established a direct relationship between abnormal thalamic rhythmicity related to Thalamo-cortical Dysrhythmia (TCD) and the occurrence of central pain. Here, this relationship has been examined using magneto-encephalographic (MEG) imaging in CRPS Type I, characterized by the absence of nerve lesions. The study addresses spontaneous MEG activity from 13 awake, adult patients (2 men, 11 women; age 15-62), with CRPS Type I of one extremity (duration range: 3months to 10years) and from 13 control subjects. All CRPS I patients demonstrated peaks in power spectrum in the delta (CRPS Type I patients presented abnormal brain activity typical of TCD, which has both diagnostic value indicating a central origin for this ailment and a potential treatment interest involving pharmacological and electrical stimulation therapies. Copyright 2010 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  2. Interaction of hyperalgesia and sensory loss in complex regional pain syndrome type I (CRPS I.

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    Volker Huge

    Full Text Available BACKGROUND: Sensory abnormalities are a key feature of Complex Regional Pain Syndrome (CRPS. In order to characterise these changes in patients suffering from acute or chronic CRPS I, we used Quantitative Sensory Testing (QST in comparison to an age and gender matched control group. METHODS: 61 patients presenting with CRPS I of the upper extremity and 56 healthy subjects were prospectively assessed using QST. The patients' warm and cold detection thresholds (WDT; CDT, the heat and cold pain thresholds (HPT; CPT and the occurrence of paradoxical heat sensation (PHS were observed. RESULTS: In acute CRPS I, patients showed warm and cold hyperalgesia, indicated by significant changes in HPT and CPT. WDT and CDT were significantly increased as well, indicating warm and cold hypoaesthesia. In chronic CRPS, thermal hyperalgesia declined, but CDT as well as WDT further deteriorated. Solely patients with acute CRPS displayed PHS. To a minor degree, all QST changes were also present on the contralateral limb. CONCLUSIONS: We propose three pathomechanisms of CRPS I, which follow a distinct time course: Thermal hyperalgesia, observed in acute CRPS, indicates an ongoing aseptic peripheral inflammation. Thermal hypoaesthesia, as detected in acute and chronic CRPS, signals a degeneration of A-delta and C-fibres, which further deteriorates in chronic CRPS. PHS in acute CRPS I indicates that both inflammation and degeneration are present, whilst in chronic CRPS I, the pathomechanism of degeneration dominates, signalled by the absence of PHS. The contralateral changes observed strongly suggest the involvement of the central nervous system.

  3. Intrinsic brain networks normalize with treatment in pediatric complex regional pain syndrome

    Science.gov (United States)

    Becerra, Lino; Sava, Simona; Simons, Laura E.; Drosos, Athena M.; Sethna, Navil; Berde, Charles; Lebel, Alyssa A.; Borsook, David

    2014-01-01

    Pediatric complex regional pain syndrome (P-CRPS) offers a unique model of chronic neuropathic pain as it either resolves spontaneously or through therapeutic interventions in most patients. Here we evaluated brain changes in well-characterized children and adolescents with P-CRPS by measuring resting state networks before and following a brief (median = 3 weeks) but intensive physical and psychological treatment program, and compared them to matched healthy controls. Differences in intrinsic brain networks were observed in P-CRPS compared to controls before treatment (disease state) with the most prominent differences in the fronto-parietal, salience, default mode, central executive, and sensorimotor networks. Following treatment, behavioral measures demonstrated a reduction of symptoms and improvement of physical state (pain levels and motor functioning). Correlation of network connectivities with spontaneous pain measures pre- and post-treatment indicated concomitant reductions in connectivity in salience, central executive, default mode and sensorimotor networks (treatment effects). These results suggest a rapid alteration in global brain networks with treatment and provide a venue to assess brain changes in CRPS pre- and post-treatment, and to evaluate therapeutic effects. PMID:25379449

  4. Sex differences in complex regional pain syndrome type I (CRPS-I) in mice.

    Science.gov (United States)

    Tang, Chaoliang; Li, Juan; Tai, Wai Lydia; Yao, Weifeng; Zhao, Bo; Hong, Junmou; Shi, Si; Wang, Song; Xia, Zhongyuan

    2017-01-01

    Sex differences have been increasingly highlighted in complex regional pain syndrome (CRPS) in clinical practice. In CRPS type I (CRPS-I), although inflammation and oxidative stress have been implicated in its pathogenesis, whether pain behavior and the underlying mechanism are sex-specific is unclear. In the present study, we sought to explore whether sex differences have an impact on inflammation, oxidative stress, and pain sensitivity in CRPS-I. Chronic post-ischemia pain (CPIP) was established in both male and female mice as an animal model of CRPS-I. Edema and mechanical allodynia of bilateral hind paws were assessed after reperfusion. Blood samples were analyzed for serum levels of oxidative stress markers and inflammatory cytokines. Both male and female mice developed edema. Male mice developed CPIP at day 3 after reperfusion; female mice developed CPIP at day 2 after reperfusion. Female mice displayed significantly earlier and higher mechanical allodynia in the ischemic hind paw, which was associated with higher serum levels of IL-2, TNF-α, isoprostanes, 8 OhdG, and malondialdehyde at day 2 after reperfusion. Moreover, female mice showed significantly lower SOD and IL-4 compared to male mice at day 2 after reperfusion. Our results indicate that sex differences in inflammatory and oxidative stress states may play a central role in the sex-specific nociceptive hypersensitivity in CRPS-I, and offer a new insight into pharmacology treatments to improve pain management with CRPS.

  5. Local cytokine changes in complex regional pain syndrome type I (CRPS I) resolve after 6 months.

    Science.gov (United States)

    Lenz, Melanie; Uçeyler, Nurcan; Frettlöh, Jule; Höffken, Oliver; Krumova, Elena K; Lissek, Silke; Reinersmann, Annika; Sommer, Claudia; Stude, Philipp; Waaga-Gasser, Ana M; Tegenthoff, Martin; Maier, Christoph

    2013-10-01

    There is evidence that inflammatory processes are involved in at least the early phase of complex regional pain syndrome (CRPS). We compared a panel of pro- and antiinflammatory cytokines in skin blister fluids and serum from patients with CRPS and patients with upper-limb pain of other origin (non-CRPS) in the early stage (CRPS patients compared to non-CRPS patients. Neither group showed side differences. After 6 months under analgesic treatment, protein levels of all measured cytokines in CRPS patients, except for IL-6, significantly changed bilaterally to the level of non-CRPS patients. These changes were not related to treatment outcome. In serum, only IL-8, TNF-α, eotaxin, MCP-1, and MIP-1β were detectable without intergroup differences. Blister fluid of CRPS patients showed a bilateral proinflammatory cytokine profile. This profile seems to be relevant only at the early stage of CRPS. Almost all measured cytokine levels were comparable to those of non-CRPS patients after 6 months of analgesic treatment and were not related to treatment outcome. Copyright © 2013 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  6. Intrinsic brain networks normalize with treatment in pediatric complex regional pain syndrome

    Directory of Open Access Journals (Sweden)

    Lino Becerra

    2014-01-01

    Full Text Available Pediatric complex regional pain syndrome (P-CRPS offers a unique model of chronic neuropathic pain as it either resolves spontaneously or through therapeutic interventions in most patients. Here we evaluated brain changes in well-characterized children and adolescents with P-CRPS by measuring resting state networks before and following a brief (median = 3 weeks but intensive physical and psychological treatment program, and compared them to matched healthy controls. Differences in intrinsic brain networks were observed in P-CRPS compared to controls before treatment (disease state with the most prominent differences in the fronto-parietal, salience, default mode, central executive, and sensorimotor networks. Following treatment, behavioral measures demonstrated a reduction of symptoms and improvement of physical state (pain levels and motor functioning. Correlation of network connectivities with spontaneous pain measures pre- and post-treatment indicated concomitant reductions in connectivity in salience, central executive, default mode and sensorimotor networks (treatment effects. These results suggest a rapid alteration in global brain networks with treatment and provide a venue to assess brain changes in CRPS pre- and post-treatment, and to evaluate therapeutic effects.

  7. Interaction of hyperalgesia and sensory loss in complex regional pain syndrome type I (CRPS I).

    Science.gov (United States)

    Huge, Volker; Lauchart, Meike; Förderreuther, Stefanie; Kaufhold, Wibke; Valet, Michael; Azad, Shahnaz Christina; Beyer, Antje; Magerl, Walter

    2008-07-23

    Sensory abnormalities are a key feature of Complex Regional Pain Syndrome (CRPS). In order to characterise these changes in patients suffering from acute or chronic CRPS I, we used Quantitative Sensory Testing (QST) in comparison to an age and gender matched control group. 61 patients presenting with CRPS I of the upper extremity and 56 healthy subjects were prospectively assessed using QST. The patients' warm and cold detection thresholds (WDT; CDT), the heat and cold pain thresholds (HPT; CPT) and the occurrence of paradoxical heat sensation (PHS) were observed. In acute CRPS I, patients showed warm and cold hyperalgesia, indicated by significant changes in HPT and CPT. WDT and CDT were significantly increased as well, indicating warm and cold hypoaesthesia. In chronic CRPS, thermal hyperalgesia declined, but CDT as well as WDT further deteriorated. Solely patients with acute CRPS displayed PHS. To a minor degree, all QST changes were also present on the contralateral limb. We propose three pathomechanisms of CRPS I, which follow a distinct time course: Thermal hyperalgesia, observed in acute CRPS, indicates an ongoing aseptic peripheral inflammation. Thermal hypoaesthesia, as detected in acute and chronic CRPS, signals a degeneration of A-delta and C-fibres, which further deteriorates in chronic CRPS. PHS in acute CRPS I indicates that both inflammation and degeneration are present, whilst in chronic CRPS I, the pathomechanism of degeneration dominates, signalled by the absence of PHS. The contralateral changes observed strongly suggest the involvement of the central nervous system.

  8. A case of complex regional pain syndrome with agnosia for object orientation.

    Science.gov (United States)

    Robinson, Gail; Cohen, Helen; Goebel, Andreas

    2011-07-01

    This systematic investigation of the neurocognitive correlates of complex regional pain syndrome (CRPS) in a single case also reports agnosia for object orientation in the context of persistent CRPS. We report a patient (JW) with severe long-standing CRPS who had no difficulty identifying and naming line drawings of objects presented in 1 of 4 cardinal orientations. In contrast, he was extremely poor at reorienting these objects into the correct upright orientation and in judging whether an object was upright or not. Moreover, JW made orientation errors when copying drawings of objects, and he also showed features of mirror reversal in writing single words and reading single letters. The findings are discussed in relation to accounts of visual processing. Agnosia for object orientation is the term for impaired knowledge of an object's orientation despite good recognition and naming of the same misoriented object. This defect has previously only been reported in patients with major structural brain lesions. The neuroanatomical correlates are discussed. The patient had no structural brain lesion, raising the possibility that nonstructural reorganisation of cortical networks may be responsible for his deficits. Other patients with CRPS may have related neurocognitive defects. Crown Copyright © 2011. Published by Elsevier B.V. All rights reserved.

  9. Impaired recognition of social emotion in patients with complex regional pain syndrome.

    Science.gov (United States)

    Shin, Na Young; Kang, Do-Hyung; Jang, Joon Hwan; Park, Soo Young; Hwang, Jae Yeon; Kim, Sung Nyun; Byun, Min Soo; Park, Hye Youn; Kim, Yong Chul

    2013-11-01

    Multiple brain areas involved in nociceptive, autonomic, and social-emotional processing are disproportionally changed in patients with complex regional pain syndrome (CRPS). Little empirical evidence is available involving social cognitive functioning in patients with chronic pain conditions. We investigated the ability of patients with CRPS to recognize the mental/emotional states of other people. Forty-three patients with CRPS and 30 healthy controls performed the Reading Mind in the Eyes Test, which consists of photos in which human eyes express various emotional and mental states. Neuropsychological tests, including the Wisconsin Card Sorting Test, the stop-signal test, and the reaction time test, were administered to evaluate other cognitive functions. Patients with CRPS were significantly less accurate at recognizing emotional states in other persons, but not on other cognitive tests, compared with control subjects. We found a significant association between the deficit in social-emotion recognition and the affective dimension of pain, whereas this deficit was not related to the sensory dimension of pain. Our findings suggest a disrupted ability to recognize others' mental/emotional states in patients with CRPS. This article demonstrated a deficit in inferring mental/emotional states of others in patients with CRPS that was related to pain affect. Our study suggests that additional interventions directed toward reducing distressful affective pain may be helpful to restore social cognitive processing in patients with CRPS. Copyright © 2013 American Pain Society. Published by Elsevier Inc. All rights reserved.

  10. Bee Venom Pharmacopuncture: An Effective Treatment for Complex Regional Pain Syndrome

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    Jong-Min Kim

    2014-12-01

    Full Text Available Objectives: Treating complex regional pain syndrome (CRPS is difficult because it still does not have a recommended therapy. A 29-year-old man was diagnosed with CRPS after surgery on his 4th and 5th left toes 7 years ago. Though he had undergone diverse pain treatment, the symptoms persisted, so he visited Dunsan Korean Medicine Hospital of Daejeon University. This case report presents results on the effect of bee venom pharmacopuncture in treating patient with CRPS. Methods: Bee venom pharmacopuncture (BVP, 0.15 to 0.4 mL dosage, was administered at GB43. The treatment was applied each week for a total 14 times. The symptoms were evaluated using a numeric rating scale (NRS and the dosage of pain medicine. Results: On the first visit, he was taking an anticonvulsant, a trycyclic antidepressant, and an analgesic. On the NRS the worst pain in the toes received a score of 8. He also complained of severe pain and hypersensitivity when the 4th and the 5th toes were touched just slightly. Other complaint included dyspepsia, rash, and depression. After treatment, on the NRS, the score for toe pain was 0, and he no longer needed to take pain medication. During the 4-months follow-up period, he has remained without pain; neither have additional symptoms appeared nor adverse events occurred. Conclusion: BVP may have potential benefits for treating patients with CRPS.

  11. Correlates and importance of neglect-like symptoms in complex regional pain syndrome.

    Science.gov (United States)

    Wittayer, Matthias; Dimova, Violeta; Birklein, Frank; Schlereth, Tanja

    2018-05-01

    Neglect-like symptoms (NLS) are frequently observed in complex regional pain syndrome (CRPS). The clinical meaning of NLS, however, is largely unknown. Therefore, this study sets out to assess the importance of NLS for patient outcome and to explore their clinical correlates. We assessed NLS in a group of 53 patients with CRPS and compared the results to 28 healthy volunteers. To define the origin of the NLS reports, we tested the subjective visual midline, performed a limb-laterality recognition test, and quantitative sensory testing. In addition, psychological and pain assessment scales were completed. Tests were analyzed with univariate and multivariate approaches. After 6 months, patients were reassessed and the influence of NLS on pain outcome was determined. Most patients reported NLS in the questionnaire, whereas subjective visual midline and limb-laterality recognition test in contrast to previous studies did not reveal perceptual disturbances. Neglect-like symptom scores were associated with pain and pain catastrophizing in acute CRPS and anxiety and thermal sensory loss in chronic CRPS. Furthermore, high NLS scores had a negative impact on pain outcome after 6 months. Our results indicate that NLS have a different meaning in acute and chronic CRPS and might be of prognostic value. Possibly, treatment should focus on reducing NLS.

  12. Evaluation of brain metabolite in patients with complex regional pain syndrome by MR spectroscopy

    International Nuclear Information System (INIS)

    Iwashita, Narihito; Fukui, Mikio; Nitta, Kazuhito; Anzawa, Noriyuki; Tomie, Hisashi; Nakanishi, Miho; Matsumoto, Tomikichi; Nosaka, Shuichi

    2010-01-01

    Recently brain imaging studies have shown that patients with chronic pain have an altered cortical processing of nociceptive inputs. We evaluated brain metabolites in patients with complex regional pain syndrome (CRPS) using MR spectroscopy. Absolute concentrations of N-acetylaspartate (NAA) and choline (Cho) were measured in anterior cingulate (ACC) and prefrontal cortices (PFC) of patients and volunteers as matched control. Psychological aspects of patients were also evaluated with Hospital Anxiety and Depression (HAD) scale, in addition to the intensity of pain by visual analog scale. In the ACC, CRPS patients had a significant decrease of NAA and a significant increase of Cho compared to the control. Furthermore, patients with anxiety scored by HAD scale had reduced NAA concentration in ACC compared to the patients without anxiety. In the PFC, there was a reduction of NAA in the patients compared with that in control. No correlation was observed between intensity of pain and these metabolites. These results suggest that metabolite changes in ACC and PFC could reflect the pathogenesis of CRPS. (author)

  13. Abnormal Brain Responses to Action Observation in Complex Regional Pain Syndrome.

    Science.gov (United States)

    Hotta, Jaakko; Saari, Jukka; Koskinen, Miika; Hlushchuk, Yevhen; Forss, Nina; Hari, Riitta

    2017-03-01

    Patients with complex regional pain syndrome (CRPS) display various abnormalities in central motor function, and their pain is intensified when they perform or just observe motor actions. In this study, we examined the abnormalities of brain responses to action observation in CRPS. We analyzed 3-T functional magnetic resonance images from 13 upper limb CRPS patients (all female, ages 31-58 years) and 13 healthy, age- and sex-matched control subjects. The functional magnetic resonance imaging data were acquired while the subjects viewed brief videos of hand actions shown in the first-person perspective. A pattern-classification analysis was applied to characterize brain areas where the activation pattern differed between CRPS patients and healthy subjects. Brain areas with statistically significant group differences (q frontal gyrus, secondary somatosensory cortex, inferior parietal lobule, orbitofrontal cortex, and thalamus. Our findings indicate that CRPS impairs action observation by affecting brain areas related to pain processing and motor control. This article shows that in CRPS, the observation of others' motor actions induces abnormal neural activity in brain areas essential for sensorimotor functions and pain. These results build the cerebral basis for action-observation impairments in CRPS. Copyright © 2016 American Pain Society. Published by Elsevier Inc. All rights reserved.

  14. Psychological distress and stressful life events in pediatric complex regional pain syndrome

    Science.gov (United States)

    Wager, Julia; Brehmer, Hannah; Hirschfeld, Gerrit; Zernikow, Boris

    2015-01-01

    BACKGROUND: There is little knowledge regarding the association between psychological factors and complex regional pain syndrome (CRPS) in children. Specifically, it is not known which factors precipitate CRPS and which result from the ongoing painful disease. OBJECTIVES: To examine symptoms of depression and anxiety as well as the experience of stressful life events in children with CRPS compared with children with chronic primary headaches and functional abdominal pain. METHODS: A retrospective chart study examined children with CRPS (n=37) who received intensive inpatient pain treatment between 2004 and 2010. They were compared with two control groups (chronic primary headaches and functional abdominal pain; each n=37), who also received intensive inpatient pain treatment. Control groups were matched with the CRPS group with regard to admission date, age and sex. Groups were compared on symptoms of depression and anxiety as well as stressful life events. RESULTS: Children with CRPS reported lower anxiety and depression scores compared with children with abdominal pain. A higher number of stressful life events before and after the onset of the pain condition was observed for children with CRPS. CONCLUSIONS: Children with CRPS are not particularly prone to symptoms of anxiety or depression. Importantly, children with CRPS experienced more stressful life events than children with chronic headaches or abdominal pain. Prospective long-term studies are needed to further explore the potential role of stressful life events in the etiology of CRPS. PMID:26035287

  15. Cerebral glucose metabolism change in patients with complex regional pain syndrome. A PET study

    International Nuclear Information System (INIS)

    Shiraishi, Satoe; Kobayashi, Hidetoshi; Nihashi, Takashi

    2006-01-01

    The aim of this study was to examine abnormalities of the central nervous system in patients with chronic pain who were diagnosed with complex regional pain syndrome (CRPS). Brain activity was assessed using 18 F-fluorodeoxyglucose positron emission tomography. The data collected from 18 patients were compared with data obtained from 13 normal age-matched controls. Our results showed that glucose metabolism was bilaterally increased in the secondary somatosensory cortex, mid-anterior cingulated cortex (ACC) or posterior cingulated cortex (PCC) (or both), parietal cortex, posterior parietal cortex (PPC), and cerebellum as well as in the right posterior insula and right thalamus in our patients. In contrast, glucose metabolism was reduced contralaterally in the dorsal prefrontal cortex and primary motor cortex. Glucose metabolism was bilaterally elevated in the mid-ACC/PCC and the PPC, which correlated with pain duration. These data suggested that glucose metabolism in the brains of patients with CRPS changes dramatically at each location. In particular, glucose metabolism was increased in the areas concerned with somatosensory perception, possibly due to continuous painful stimulation. (author)

  16. Highly restricted deletion of the SNORD116 region is implicated in Prader–Willi Syndrome

    Science.gov (United States)

    Bieth, Eric; Eddiry, Sanaa; Gaston, Véronique; Lorenzini, Françoise; Buffet, Alexandre; Conte Auriol, Françoise; Molinas, Catherine; Cailley, Dorothée; Rooryck, Caroline; Arveiler, Benoit; Cavaillé, Jérome; Salles, Jean Pierre; Tauber, Maïthé

    2015-01-01

    The SNORD116 locus lies in the 15q11-13 region of paternally expressed genes implicated in Prader–Willi Syndrome (PWS), a complex disease accompanied by obesity and severe neurobehavioural disturbances. Cases of PWS patients with a deletion encompassing the SNORD116 gene cluster, but preserving the expression of flanking genes, have been described. We report a 23-year-old woman who presented clinical criteria of PWS, including the behavioural and nutritional features, obesity, developmental delay and endocrine dysfunctions with hyperghrelinemia. We found a paternally transmitted highly restricted deletion of the SNORD116 gene cluster, the shortest described to date (118 kb). This deletion was also present in the father. This finding in a human case strongly supports the current hypothesis that lack of the paternal SNORD116 gene cluster has a determinant role in the pathogenesis of PWS. Moreover, targeted analysis of the SNORD116 gene cluster, complementary to SNRPN methylation analysis, should be carried out in subjects with a phenotype suggestive of PWS. PMID:24916642

  17. Highly restricted deletion of the SNORD116 region is implicated in Prader-Willi Syndrome.

    Science.gov (United States)

    Bieth, Eric; Eddiry, Sanaa; Gaston, Véronique; Lorenzini, Françoise; Buffet, Alexandre; Conte Auriol, Françoise; Molinas, Catherine; Cailley, Dorothée; Rooryck, Caroline; Arveiler, Benoit; Cavaillé, Jérome; Salles, Jean Pierre; Tauber, Maïthé

    2015-02-01

    The SNORD116 locus lies in the 15q11-13 region of paternally expressed genes implicated in Prader-Willi Syndrome (PWS), a complex disease accompanied by obesity and severe neurobehavioural disturbances. Cases of PWS patients with a deletion encompassing the SNORD116 gene cluster, but preserving the expression of flanking genes, have been described. We report a 23-year-old woman who presented clinical criteria of PWS, including the behavioural and nutritional features, obesity, developmental delay and endocrine dysfunctions with hyperghrelinemia. We found a paternally transmitted highly restricted deletion of the SNORD116 gene cluster, the shortest described to date (118 kb). This deletion was also present in the father. This finding in a human case strongly supports the current hypothesis that lack of the paternal SNORD116 gene cluster has a determinant role in the pathogenesis of PWS. Moreover, targeted analysis of the SNORD116 gene cluster, complementary to SNRPN methylation analysis, should be carried out in subjects with a phenotype suggestive of PWS.

  18. Objective sensory evaluation of the spread of complex regional pain syndrome.

    Science.gov (United States)

    Edinger, Lara; Schwartzman, Robert J; Ahmad, Ayesha; Erwin, Kirsten; Alexander, Guillermo M

    2013-01-01

    The spread of complex regional pain syndrome (CRPS) has been well documented. Many severe refractory long-standing patients have total body pain (TBP) that evolved from a single extremity injury. The purpose of this study was to document by objective sensory threshold testing the extent of body area involvement in 20 long-standing patients with CRPS who have TBP. A comparison of sensory threshold testing parameters between 20 long-standing refractory patients with CRPS who have TBP versus 10 healthy participants. Twenty patients with CRPS who stated that they suffered from total body pain were chosen from the Drexel University College of Medicine CRPS database. They were compared to 10 healthy participants that were age and gender matched to the patients with CRPS. The sensory parameters tested were: skin temperature; static and mechanical allodynia; thermal allodynia; mechanical hyperalgesia; after sensations following all sensory tests. The sites chosen for testing in the patients with CRPS were the most painful area in each of 8 body regions that comprised the total body area. Five patients with CRPS had signs of CRPS over 100% of their body (20%). One patient had pain over 87% and another had pain over 90% of their body area. The average percentage of body involvement was 62% (range 37% - 100%). All patients with CRPS had at least one sensory parameter abnormality in all body regions. All patients with CRPS had lower pain thresholds for static allodynia in all body areas, while 50% demonstrated a lower threshold for dynamic allodynia in all body regions compared to the healthy participants. Cold allodynia had a higher median pain rating on the Likert pain scale in all body areas versus healthy participants except for the chest, abdomen, and back. Eighty-five percent of the patients with CRPS had a significantly lower pain threshold for mechanical hyperalgesia in all body areas compared to the healthy participants. After sensations occurred after all sensory

  19. Central nervous system PET-CT imaging reveals regional impairments in pediatric patients with Wolfram syndrome.

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    Agnieszka Zmyslowska

    Full Text Available Wolfram syndrome (WFS is inherited as an autosomal recessive disease with main clinical features of diabetes mellitus, optic atrophy, diabetes insipidus and deafness. However, various neurological defects may also be detected. The aim of this study was to evaluate aspects of brain structure and function using PET-CT (positron emission tomography and computed tomography and MRI (magnetic resonance imaging in pediatric patients with WFS. Regional changes in brain glucose metabolism were measured using standardized uptake values (SUVs based on images of (18F fluorodeoxyglucose (FDG uptake in 7 WFS patients aged 10.1-16.0 years (mean 12.9±2.4 and in 20 healthy children aged 3-17.9 years (mean 12.8±4.1. In all patients the diagnosis of WFS was confirmed by DNA sequencing of the WFS1 gene. Hierarchical clustering showed remarkable similarities of glucose uptake patterns among WFS patients and their differences from the control group. SUV data were subsequently standardized for age groups 13 years old to account for developmental differences. Reduced SUVs in WFS patients as compared to the control group for the bilateral brain regions such as occipital lobe (-1.24±1.20 vs. -0.13±1.05; p = 0.028 and cerebellum (-1.11±0.69 vs. -0.204±1.00; p = 0.036 were observed and the same tendency for cingulate (-1.13±1.05 vs. -0.15±1.12; p = 0.056, temporal lobe (-1.10±0.98 vs. -0.15±1.10; p = 0.057, parietal lobe (-1.06±1.20 vs. -0.08±1.08; p = 0.058, central region (-1.01±1.04 vs. -0.09±1.06; p = 0.060, basal ganglia (-1.05±0.74 vs. -0.20±1.07; p = 0.066 and mesial temporal lobe (-1.06±0.82 vs. -0.26±1.08; p = 0.087 was also noticed. After adjusting for multiple hypothesis testing, the differences in glucose uptake were non-significant. For the first time, regional differences in brain glucose metabolism among patients with WFS were shown using PET-CT imaging.

  20. Complex Regional Pain Syndrome Type I Affects Brain Structure in Prefrontal and Motor Cortex

    Science.gov (United States)

    Pleger, Burkhard; Draganski, Bogdan; Schwenkreis, Peter; Lenz, Melanie; Nicolas, Volkmar; Maier, Christoph; Tegenthoff, Martin

    2014-01-01

    The complex regional pain syndrome (CRPS) is a rare but debilitating pain disorder that mostly occurs after injuries to the upper limb. A number of studies indicated altered brain function in CRPS, whereas possible influences on brain structure remain poorly investigated. We acquired structural magnetic resonance imaging data from CRPS type I patients and applied voxel-by-voxel statistics to compare white and gray matter brain segments of CRPS patients with matched controls. Patients and controls were statistically compared in two different ways: First, we applied a 2-sample ttest to compare whole brain white and gray matter structure between patients and controls. Second, we aimed to assess structural alterations specifically of the primary somatosensory (S1) and motor cortex (M1) contralateral to the CRPS affected side. To this end, MRI scans of patients with left-sided CRPS (and matched controls) were horizontally flipped before preprocessing and region-of-interest-based group comparison. The unpaired ttest of the “non-flipped” data revealed that CRPS patients presented increased gray matter density in the dorsomedial prefrontal cortex. The same test applied to the “flipped” data showed further increases in gray matter density, not in the S1, but in the M1 contralateral to the CRPS-affected limb which were inversely related to decreased white matter density of the internal capsule within the ipsilateral brain hemisphere. The gray-white matter interaction between motor cortex and internal capsule suggests compensatory mechanisms within the central motor system possibly due to motor dysfunction. Altered gray matter structure in dorsomedial prefrontal cortex may occur in response to emotional processes such as pain-related suffering or elevated analgesic top-down control. PMID:24416397

  1. Complex regional pain syndrome type I affects brain structure in prefrontal and motor cortex.

    Directory of Open Access Journals (Sweden)

    Burkhard Pleger

    Full Text Available The complex regional pain syndrome (CRPS is a rare but debilitating pain disorder that mostly occurs after injuries to the upper limb. A number of studies indicated altered brain function in CRPS, whereas possible influences on brain structure remain poorly investigated. We acquired structural magnetic resonance imaging data from CRPS type I patients and applied voxel-by-voxel statistics to compare white and gray matter brain segments of CRPS patients with matched controls. Patients and controls were statistically compared in two different ways: First, we applied a 2-sample ttest to compare whole brain white and gray matter structure between patients and controls. Second, we aimed to assess structural alterations specifically of the primary somatosensory (S1 and motor cortex (M1 contralateral to the CRPS affected side. To this end, MRI scans of patients with left-sided CRPS (and matched controls were horizontally flipped before preprocessing and region-of-interest-based group comparison. The unpaired ttest of the "non-flipped" data revealed that CRPS patients presented increased gray matter density in the dorsomedial prefrontal cortex. The same test applied to the "flipped" data showed further increases in gray matter density, not in the S1, but in the M1 contralateral to the CRPS-affected limb which were inversely related to decreased white matter density of the internal capsule within the ipsilateral brain hemisphere. The gray-white matter interaction between motor cortex and internal capsule suggests compensatory mechanisms within the central motor system possibly due to motor dysfunction. Altered gray matter structure in dorsomedial prefrontal cortex may occur in response to emotional processes such as pain-related suffering or elevated analgesic top-down control.

  2. Metabolic syndrome according to different definitions in a rapidly developing country of the African region

    Directory of Open Access Journals (Sweden)

    Paccaud Fred

    2008-09-01

    Full Text Available Abstract Aims We examined, in a country of the African region, i the prevalence of the metabolic syndrome (MetS according to three definitions (ATP, WHO and IDF; ii the distribution of the MetS criteria; iii the level of agreement between these three definitions and iv we also examined these issues upon exclusion of people with diabetes. Methods We conducted an examination survey on a sample representative of the general population aged 25–64 years in the Seychelles (Indian Ocean, African region, attended by 1255 participants (participation rate of 80.3%. Results The prevalence of MetS increased markedly with age. According to the ATP, WHO and IDF definitions, the prevalence of MetS was, respectively, 24.0%, 25.0%, 25.1% in men and 32.2%, 24.6%, 35.4% in women. Approximately 80% of participants with diabetes also had MetS and the prevalence of MetS was approximately 7% lower upon exclusion of diabetic individuals. High blood pressure and adiposity were the criteria found most frequently among MetS holders irrespective of the MetS definitions. Among people with MetS based on any of the three definitions, 78% met both ATP and IDF criteria, 67% both WHO and IDF criteria, 54% both WHO and ATP criteria and only 37% met all three definitions. Conclusion We identified a high prevalence of MetS in this population in epidemiological transition. The prevalence of MetS decreased by approximately 32% upon exclusion of persons with diabetes. Because of limited agreement between the MetS definitions, the fairly similar proportions of MetS based on any of the three MetS definitions classified, to a substantial extent, different subjects as having MetS.

  3. Analysis of gene expression profile microarray data in complex regional pain syndrome.

    Science.gov (United States)

    Tan, Wulin; Song, Yiyan; Mo, Chengqiang; Jiang, Shuangjian; Wang, Zhongxing

    2017-09-01

    The aim of the present study was to predict key genes and proteins associated with complex regional pain syndrome (CRPS) using bioinformatics analysis. The gene expression profiling microarray data, GSE47603, which included peripheral blood samples from 4 patients with CRPS and 5 healthy controls, was obtained from the Gene Expression Omnibus (GEO) database. The differentially expressed genes (DEGs) in CRPS patients compared with healthy controls were identified using the GEO2R online tool. Functional enrichment analysis was then performed using The Database for Annotation Visualization and Integrated Discovery online tool. Protein‑protein interaction (PPI) network analysis was subsequently performed using Search Tool for the Retrieval of Interaction Genes database and analyzed with Cytoscape software. A total of 257 DEGs were identified, including 243 upregulated genes and 14 downregulated ones. Genes in the human leukocyte antigen (HLA) family were most significantly differentially expressed. Enrichment analysis demonstrated that signaling pathways, including immune response, cell motion, adhesion and angiogenesis were associated with CRPS. PPI network analysis revealed that key genes, including early region 1A binding protein p300 (EP300), CREB‑binding protein (CREBBP), signal transducer and activator of transcription (STAT)3, STAT5A and integrin α M were associated with CRPS. The results suggest that the immune response may therefore serve an important role in CRPS development. In addition, genes in the HLA family, such as HLA‑DQB1 and HLA‑DRB1, may present potential biomarkers for the diagnosis of CRPS. Furthermore, EP300, its paralog CREBBP, and the STAT family genes, STAT3 and STAT5 may be important in the development of CRPS.

  4. Critical appraisal of ticagrelor in the management of acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Nawarskas JJ

    2011-12-01

    Full Text Available James J Nawarskas, Stanley S SnowdenUniversity of New Mexico College of Pharmacy, Albuquerque, NM, USAAbstract: Ticagrelor is a novel P2Y12 receptor antagonist which, like clopidogrel and prasugrel, functions by blocking adenosine diphosphate-mediated platelet aggregation. However, unlike the aforementioned agents, the binding of ticagrelor to this receptor is reversible. Ticagrelor is also believed to mediate some of its beneficial effects by augmenting the effects of adenosine, which is another unique pharmacologic property of this drug. In terms of antiplatelet effect, ticagrelor is more potent than clopidogrel and produces a faster and stronger inhibition of platelet aggregation. This may also be an advantage of ticagrelor over prasugrel, but this has not been adequately studied. Due to the reversible nature of the binding of ticagrelor to the platelet receptor, ticagrelor has a relatively fast offset of effect, with platelet aggregation approaching pretreatment levels about 3 days after discontinuation of therapy. This has advantages in patients requiring invasive procedures, but also makes medication adherence very important in order to be able to maintain an effective antiplatelet effect. Ticagrelor has been shown to be clinically superior to clopidogrel when given to patients with an acute coronary syndrome, resulting in significantly lower rates of myocardial infarction and vascular death. However, ticagrelor is indicated to be administered with aspirin, and the clinical benefits of ticagrelor may be less when daily dosages of aspirin exceed 100 mg. As expected, bleeding is the most common adverse effect with ticagrelor, although it occurs at rates comparable with those seen for clopidogrel with the exception of noncoronary artery bypass graft-related major bleeding and fatal intracranial bleeds, the latter of which occurs only rarely. Dyspnea is another common adverse effect with ticagrelor, although this is usually not severe and

  5. Potential Risk Factors for the Onset of Complex Regional Pain Syndrome Type 1: A Systematic Literature Review

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    Tracey Pons

    2015-01-01

    Full Text Available Anaesthetists in the acute and chronic pain teams are often involved in treating Complex Regional Pain Syndromes. Current literature about the risk factors for the onset of Complex Regional Pain Syndrome Type 1 (CRPS 1 remains sparse. This syndrome has a low prevalence, a highly variable presentation, and no gold standard for diagnosis. In the research setting, the pathogenesis of the syndrome continues to be elusive. There is a growing body of literature that addresses efficacy of a wide range of interventions as well as the likely mechanisms that contribute to the onset of CRPS 1. The objective for this systematic search of the literature focuses on determining the potential risk factors for the onset of CRPS 1. Eligible articles were analysed, dated 1996 to April 2014, and potential risk factors for the onset of CRPS 1 were identified from 10 prospective and 6 retrospective studies. Potential risk factors for the onset of CRPS 1 were found to include being female, particularly postmenopausal female, ankle dislocation or intra-articular fracture, immobilisation, and a report of higher than usual levels of pain in the early phases of trauma. It is not possible to draw definite conclusions as this evidence is heterogeneous and of mixed quality, relevance, and weighting strength against bias and has not been confirmed across multiple trials or in homogenous studies.

  6. Water Information System Platforms Addressing Critical Societal Needs in the Mena Region

    Science.gov (United States)

    Habib, Shahid; Kfouri, Claire; Peters, Mark

    2012-01-01

    The MENA region includes 18 countries, the occupied Palestinian territories and Western Sahara. However, the region of interest for this study has a strategic interest in countries adjacent to the Mediterranean Sea, which includes, Morocco, Tunisia, Egypt, Lebanon and Jordan. The 90% of the water in the MENA region is used for the agriculture use. By the end of this century. this region is projected to experience an increase of 3 C to 5 C in mean temperatures and a 20% decline in precipitation (lPCC, 2007). Due to lower precipitation, water run-off is projected to drop by 20% to 30% in most of MENA by 2050 Reduced stream flow and groundwater recharge might lead to a reduction in water supply of 10% or greater by 2050. Therefore, per IPCC projections in temperature rise and precipitation decline in the region, the scarcity of water will become more acute with population growth, and rising demand of food in the region. Additionally, the trans boundary water issues will continue to plague the region in terms of sharing data for better management of water resources. Such pressing issues have brought The World Bank, USAID and NASA to jointly collaborate for establishing integrated, modern, up to date NASA developed capabilities for countries in the MENA region for addressing water resource issues and adapting to climate change impacts for improved decision making and societal benefit. This initiative was launched in October 2011 and is schedule to be completed by the end of2015.

  7. Refinement of the critical 2p25.3 deletion region

    DEFF Research Database (Denmark)

    De Rocker, Nina; Vergult, Sarah; Koolen, David

    2015-01-01

    PURPOSE: Submicroscopic deletions of chromosome band 2p25.3 are associated with intellectual disability and/or central obesity. Although MYT1L is believed to be a critical gene responsible for intellectual disability, so far no unequivocal data have confirmed this hypothesis. METHODS: In this study...

  8. Electric conductivity of alkali metal vapors in the region of critical point

    International Nuclear Information System (INIS)

    Likal'ter, A.A.

    1982-01-01

    A behaviour of alkali metal conductivity in the vicinity of a critical point has been analyzed on the base of deVeloped representations on a vapor state. A phenomenological conductivity theory has been developed, which is in a good agreement with experimental data obtained

  9. Load-Unload Response Ratio and Accelerating Moment/Energy Release Critical Region Scaling and Earthquake Prediction

    Science.gov (United States)

    Yin, X. C.; Mora, P.; Peng, K.; Wang, Y. C.; Weatherley, D.

    The main idea of the Load-Unload Response Ratio (LURR) is that when a system is stable, its response to loading corresponds to its response to unloading, whereas when the system is approaching an unstable state, the response to loading and unloading becomes quite different. High LURR values and observations of Accelerating Moment/Energy Release (AMR/AER) prior to large earthquakes have led different research groups to suggest intermediate-term earthquake prediction is possible and imply that the LURR and AMR/AER observations may have a similar physical origin. To study this possibility, we conducted a retrospective examination of several Australian and Chinese earthquakes with magnitudes ranging from 5.0 to 7.9, including Australia's deadly Newcastle earthquake and the devastating Tangshan earthquake. Both LURR values and best-fit power-law time-to-failure functions were computed using data within a range of distances from the epicenter. Like the best-fit power-law fits in AMR/AER, the LURR value was optimal using data within a certain epicentral distance implying a critical region for LURR. Furthermore, LURR critical region size scales with mainshock magnitude and is similar to the AMR/AER critical region size. These results suggest a common physical origin for both the AMR/AER and LURR observations. Further research may provide clues that yield an understanding of this mechanism and help lead to a solid foundation for intermediate-term earthquake prediction.

  10. India’s Look/Act East Policy and the Northeast Region: A Critical Perspective

    Directory of Open Access Journals (Sweden)

    Professor Hiranya K Nath

    2017-11-01

    Full Text Available India’s Look East Policy (LEP signifies a strategic shift in its international political, economic, and military relationships. Regional integration of its Northeast Region (NER with the countries in East, Southeast and South Asia may potentially generate economic dividends to the region. However, there are formidable challenges in realizing the potentials. The proposed infrastructure projects, if completed with no further delay, will go a long way in improving connectivity with the neighbouring countries. However, improving connectivity within the region and with the rest of the country is also very important. Further, it would require a comprehensive long-term plan with well-defined projects for developing industries and services including education, health and tourism. Building infrastructure, ensuring socio-political stability and ecological balance, and improving the quality of institutions would be a major part of this plan.

  11. A novel missense mutation (G43S) in the switch I region of Rab27A causing Griscelli syndrome

    DEFF Research Database (Denmark)

    Westbroek, W.; Tuchman, M.; Tinloy, B.

    2008-01-01

    The autosomal recessive Griscelli syndrome type II (GSII) is caused by mutations in the RAB27A gene. Typical clinical features include immunological impairment, silver-gray scalp hair, eyelashes and eyebrows and hypomelanosis of the skin. Rabs help determine the specificity of membrane trafficking......-immunoprecipitation studies showed that Rab27A(G43S) fails to interact with its effector Melanophilin, indicating that the switch I region functions in the recruitment of Rab effector proteins Udgivelsesdato: 2008/6...

  12. Negative Symptom Dimensions of the Positive and Negative Syndrome Scale Across Geographical Regions: Implications for Social, Linguistic, and Cultural Consistency

    OpenAIRE

    Khan, Anzalee; Liharska, Lora; Harvey, Philip D.; Atkins, Alexandra; Ulshen, Daniel; Keefe, Richard S.E.

    2017-01-01

    Objective: Recognizing the discrete dimensions that underlie negative symptoms in schizophrenia and how these dimensions are understood across localities might result in better understanding and treatment of these symptoms. To this end, the objectives of this study were to 1) identify the Positive and Negative Syndrome Scale negative symptom dimensions of expressive deficits and experiential deficits and 2) analyze performance on these dimensions over 15 geographical regions to determine whet...

  13. Association of non-traumatic complex regional pain syndrome with adenocarcinoma lung on 99mTc-MDP bone scan

    International Nuclear Information System (INIS)

    Damle, Nishikant A.; Tripathi, Madhavi; Singhal, Abhinav; Bal, Chandrasekhar; Praveen Kumar; Kandasamy, Devasenathipathi; Jana, Manisha

    2012-01-01

    Complex regional pain syndrome (CRPS) is usually associated with trauma. Rarely, it may be seen in association with malignancies. We present here the bone scan and X-ray findings in the case of a 56-year-male-patient with adenocarcinoma lung who also had non-traumatic CRPS without involvement of the stellate ganglion. The case highlights the fact that spontaneous development of reflex sympathetic dystrophy may be associated with a neoplastic etiology. (author)

  14. Fine Mapping Suggests that the Goat Polled Intersex Syndrome and the Human Blepharophimosis Ptosis Epicanthus Syndrome Map to a 100-kb Homologous Region

    OpenAIRE

    Schibler, Laurent; Cribiu, Edmond P.; Oustry-Vaiman, Anne; Furet, Jean-Pierre; Vaiman, Daniel

    2000-01-01

    To clone the goat Polled Intersex Syndrome (PIS) gene(s), a chromosome walk was performed from six entry points at 1q43. This enabled 91 BACs to be recovered from a recently constructed goat BAC library. Six BAC contigs of goat chromosome 1q43 (ICC1–ICC6) were thus constructed covering altogether 4.5 Mb. A total of 37 microsatellite sequences were isolated from this 4.5-Mb region (16 in this study), of which 33 were genotyped and mapped. ICC3 (1500 kb) was shown by genetic analysis to encompa...

  15. A contribution to genetic etiology of complex regional pain syndrome type I (algodystropy syndrome) based on quantitative analysis of digitopalmar dermatoglyphics in sixty men.

    Science.gov (United States)

    Cvjeticanin, Miljenko; Jajić, Zrinka; Jajić, Ivo

    2005-01-01

    The patterns of the ridges of the skin of the fingers and palms were determined in sixty men with complex regional pain syndrome (type I) as a measure of disease prevention. The study included 25 dermatoglyphic traits: number of epidermal ridges on all ten fingers; their sum for five and ten fingers; four traits on both palms, i.e. between a-b, b-c and c-d triradii; atd angles: and their bilateral sum. The data obtained were compared with those recorded in a control group of 200 pairs of imprints of phenotipycally healthy male adults from the Zagreb area. Statistically significant difference from control values were found in 12 dermatoglyphic variables, including an increased sum of ridges on nine fingers (except for left second finger pad), and total sum for five and ten fingers. These findings suggested the polygenic system responsible for development of dermatoglyphics to be identical with some polygenic loci for the onset of algodystrophy syndrome, which might prove useful in disease prevention (e.g., taking fingerprints following a trauma and before rehabilitation), and to facilitate identification of risk groups, and thus the treatment for this longterm and yet obscure syndrome.

  16. Overlapping hotspots in CDRs are critical sites for V region diversification.

    Science.gov (United States)

    Wei, Lirong; Chahwan, Richard; Wang, Shanzhi; Wang, Xiaohua; Pham, Phuong T; Goodman, Myron F; Bergman, Aviv; Scharff, Matthew D; MacCarthy, Thomas

    2015-02-17

    Activation-induced deaminase (AID) mediates the somatic hypermutation (SHM) of Ig variable (V) regions that is required for the affinity maturation of the antibody response. An intensive analysis of a published database of somatic hypermutations that arose in the IGHV3-23*01 human V region expressed in vivo by human memory B cells revealed that the focus of mutations in complementary determining region (CDR)1 and CDR2 coincided with a combination of overlapping AGCT hotspots, the absence of AID cold spots, and an abundance of polymerase eta hotspots. If the overlapping hotspots in the CDR1 or CDR2 did not undergo mutation, the frequency of mutations throughout the V region was reduced. To model this result, we examined the mutation of the human IGHV3-23*01 biochemically and in the endogenous heavy chain locus of Ramos B cells. Deep sequencing revealed that IGHV3-23*01 in Ramos cells accumulates AID-induced mutations primarily in the AGCT in CDR2, which was also the most frequent site of mutation in vivo. Replacing the overlapping hotspots in CDR1 and CDR2 with neutral or cold motifs resulted in a reduction in mutations within the modified motifs and, to some degree, throughout the V region. In addition, some of the overlapping hotspots in the CDRs were at sites in which replacement mutations could change the structure of the CDR loops. Our analysis suggests that the local sequence environment of the V region, and especially of the CDR1 and CDR2, is highly evolved to recruit mutations to key residues in the CDRs of the IgV region.

  17. A Novel Compound Analgesic Cream (Ketamine, Pentoxifylline, Clonidine, DMSO) for Complex Regional Pain Syndrome Patients.

    Science.gov (United States)

    Russo, Marc A; Santarelli, Danielle M

    2016-01-01

    Evidence suggests that complex regional pain syndrome (CRPS) is a manifestation of microvascular dysfunction. Topical combinations of α2-adrenergic receptor agonists or nitric oxide donors with phosphodiesterase or phosphatidic acid inhibitors formulated to treat microvascular dysfunction have been shown to reduce allodynia in a rat model of CRPS-I. Driven by these findings, we assessed the outcomes of CRPS patients treated with a compound analgesic cream (CAC) consisting of ketamine 10%, pentoxifylline 6%, clonidine 0.2%, and dimethyl sulfoxide 6% to 10%. An audit was conducted on 13 CRPS patients who trialed the CAC. A detailed report was compiled for each patient which comprised baseline characteristics, including CRPS description, previous treatments, and pain scores (numerical pain rating scale; 0 to 10). Recorded outcomes consisted of pain scores, descriptive outcomes, and concurrent medications/treatments, for which basic analysis was performed to determine the effectiveness of the CAC. Case reports are presented for 3 patients with varying outcomes. Nine patients (69%) reported pain/symptom reduction (4.4 ± 2.1 vs. 6.3 ± 1.9) with use of the CAC. Six patients reported sustained benefits after 2 months of CAC use, and 2 patients reported complete resolution of pain/symptoms: one had early CRPS-I and the other received a partial CRPS diagnosis. An otherwise medication refractory and intolerant patient found partial benefit with the CAC. These results demonstrate promise for this topical combination as a useful treatment in multimodal therapy for patients with CRPS, with the potential to resolve pain/symptoms in early CRPS patients. © 2015 World Institute of Pain.

  18. Complex Regional Pain Syndrome following an Episode of Herpes Zoster: A Case Report.

    Science.gov (United States)

    Marrero, Christopher E; Mclean, Neuyen; Varnado, Keyana

    2017-01-01

    Complex regional pain syndrome (CRPS) is characterized by searing pain, hyperalgesia, edema, allodynia, and skin changes. CRPS may be difficult to diagnose and to treat given poorly understood mechanisms as well as its presentation of symptoms that may mimic common conditions such as joint stiffness in this condition as well as rheumatoid arthritis. A 71-year-old female presented to our clinic post shingles of the right upper extremity. We diagnosed her with CRPS based on the Budapest diagnostic criteria and the clinical findings of pain and decreased the range of motion along with edema, hypersensitivity, discoloration and allodynia of the right thumb and index finger. She was treated with vitamin C as well as gabapentin and physical therapy. The patient was unable to go consistently to physical therapy due to insurance limitations, and we found no clinical benefit of vitamin C in reducing her symptoms. She was lost to follow-up during her treatment but re-emerged at 21 months. At that time she reported, she was largely unchanged in regards to her right-hand symptoms but did believe the gabapentin was helpful and still continued to take 300 mg daily. This case report highlights the usefulness of the Budapest diagnostic criteria to make the diagnosis of CRPS when associated with shingles, which can cause long-term pain and mimic some findings. Prompt diagnosis is important, as recovery typically extends beyond 6 months; our patient still reported continued symptoms at 21 months post initial presentation. Our primary treatment plan was physical therapy, which she discontinued due to insurance limitations. We recommend that patients, physicians, and third-party payers work together to extend access to physical therapy. More investigation is warranted regarding symptomatic treatment, as we found limited clinical benefit of gabapentin and vitamin C.

  19. Pain increases during sympathetic arousal in patients with complex regional pain syndrome.

    Science.gov (United States)

    Drummond, P D; Finch, P M; Skipworth, S; Blockey, P

    2001-10-09

    To investigate the effect of sympathetic arousal on pain and vasomotor responses in healthy control subjects and patients with complex regional pain syndrome (CRPS), and to determine whether pain increases in patients with particular symptoms. In experiments 1 and 2, capsaicin was applied to the forearm of 24 healthy subjects to induce thermal hyperalgesia. Vascular responses were monitored and subjects rated thermal hyperalgesia before and after being startled (experiment 1), and before, during, and after mental arithmetic, breath holding, forehead cooling, the Valsalva maneuver, and a cold pressor test in experiment 2. In a third experiment, sensitivity to heat, cold, and mechanical stimulation was investigated in 61 patients with CRPS. Pain ratings and vascular and electrodermal responses were recorded after patients were startled and during forehead cooling. In experiment 1, thermal hyperalgesia decreased in healthy control subjects after they were startled, and digital blood vessels constricted symmetrically. In experiment 2, thermal hyperalgesia decreased during and after other forms of sympathetic arousal. However, in experiment 3, ratings of clinical pain increased during forehead cooling or after being startled in over 70% of patients with CRPS. Pain increased most consistently during forehead cooling in patients with cold allodynia or punctate allodynia. Digital blood vessels constricted more intensely on the symptomatic than the nonsymptomatic side in patients with CRPS during sympathetic arousal. Normal inhibitory influences on pain during sympathetic arousal are compromised in the majority of patients with CRPS. The augmented vasoconstrictor response in the symptomatic limb during sympathetic arousal is consistent with adrenergic supersensitivity. An adrenergic sensitivity in nociceptive afferents might contribute to pain and hyperalgesia during sympathetic arousal in certain patients with CRPS.

  20. Selective Fiber Degeneration in the Peripheral Nerve of a Patient With Severe Complex Regional Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Adrien Yvon

    2018-04-01

    Full Text Available Aims: Complex regional pain syndrome (CRPS is characterized by chronic debilitating pain disproportional to the inciting event and accompanied by motor, sensory, and autonomic disturbances. The pathophysiology of CRPS remains elusive. An exceptional case of severe CRPS leading to forearm amputation provided the opportunity to examine nerve histopathological features of the peripheral nerves.Methods: A 35-year-old female developed CRPS secondary to low voltage electrical injury. The CRPS was refractory to medical therapy and led to functional loss of the forelimb, repeated cutaneous wound infections leading to hospitalization. Specifically, the patient had exhausted a targeted conservative pain management programme prior to forearm amputation. Radial, median, and ulnar nerve specimens were obtained from the amputated limb and analyzed by light and transmission electron microscopy (TEM.Results: All samples showed features of selective myelinated nerve fiber degeneration (47–58% of fibers on electron microscopy. Degenerating myelinated fibers were significantly larger than healthy fibers (p < 0.05, and corresponded to the larger Aα fibers (motor/proprioception whilst smaller Aδ (pain/temperature fibers were spared. Groups of small unmyelinated C fibers (Remak bundles also showed evidence of degeneration in all samples.Conclusions: We are the first to show large fiber degeneration in CRPS using TEM. Degeneration of Aα fibers may lead to an imbalance in nerve signaling, inappropriately triggering the smaller healthy Aδ fibers, which transmit pain and temperature. These findings suggest peripheral nerve degeneration may play a key role in CRPS. Improved knowledge of pathogenesis will help develop more targeted treatments.

  1. Children and adolescents with complex regional pain syndrome: More psychologically distressed than other children in pain?

    Science.gov (United States)

    Logan, Deirdre E; Williams, Sara E; Carullo, Veronica P; Claar, Robyn Lewis; Bruehl, Stephen; Berde, Charles B

    2013-01-01

    BACKGROUND Historically, in both adult and pediatric populations, a lack of knowledge regarding complex regional pain syndrome (CRPS) and absence of clear diagnostic criteria have contributed to the view that this is a primarily psychiatric condition. OBJECTIVE: To test the hypothesis that children with CRPS are more functionally disabled, have more pain and are more psychologically distressed than children with other pain conditions. METHODS: A total of 101 children evaluated in a tertiary care pediatric pain clinic who met the International Association for the Study of Pain consensus diagnostic criteria for CRPS participated in the present retrospective study. Comparison groups included 103 children with abdominal pain, 291 with headache and 119 with back pain. Children and parents completed self-report questionnaires assessing disability, somatization, pain coping, depression, anxiety and school attendance. RESULTS: Children with CRPS reported higher pain intensity and more recent onset of pain at the initial tertiary pain clinic evaluation compared with children with other chronic pain conditions. They reported greater functional disability and more somatic symptoms than children with headaches or back pain. Scores on measures of depression and anxiety were within normal limits and similar to those of children in other pain diagnostic groups. CONCLUSIONS: As a group, clinic-referred children with CRPS may be more functionally impaired and experience more somatic symptoms compared with children with other pain conditions. However, overall psychological functioning as assessed by self-report appears to be similar to that of children with other chronic pain diagnoses. Comprehensive assessment using a biopsychosocial framework is essential to understanding and appropriately treating children with symptoms of CRPS. PMID:23662291

  2. Medical history and the onset of complex regional pain syndrome (CRPS).

    Science.gov (United States)

    de Mos, M; Huygen, F J P M; Dieleman, J P; Koopman, J S H A; Stricker, B H Ch; Sturkenboom, M C J M

    2008-10-15

    Knowledge concerning the medical history prior to the onset of complex regional pain syndrome (CRPS) might provide insight into its risk factors and potential underlying disease mechanisms. To evaluate prior to CRPS medical conditions, a case-control study was conducted in the Integrated Primary Care Information (IPCI) project, a general practice (GP) database in the Netherlands. CRPS patients were identified from the records and validated through examination by the investigator (IASP criteria) or through specialist confirmation. Cases were matched to controls on age, gender and injury type. All diagnoses prior to the index date were assessed by manual review of the medical records. Some pre-specified medical conditions were studied for their association with CRPS, whereas all other diagnoses, grouped by pathogenesis, were tested in a hypothesis-generating approach. Of the identified 259 CRPS patients, 186 cases (697 controls) were included, based on validation by the investigator during a visit (102 of 134 visited patients) or on specialist confirmation (84 of 125 unvisited patients). A medical history of migraine (OR: 2.43, 95% CI: 1.18-5.02) and osteoporosis (OR: 2.44, 95% CI: 1.17-5.14) was associated with CRPS. In a recent history (1-year before CRPS), cases had more menstrual cycle-related problems (OR: 2.60, 95% CI: 1.16-5.83) and neuropathies (OR: 5.7; 95% CI: 1.8-18.7). In a sensitivity analysis, including only visited cases, asthma (OR: 3.0; 95% CI: 1.3-6.9) and CRPS were related. Psychological factors were not associated with CRPS onset. Because of the hypothesis-generating character of this study, the findings should be confirmed by other studies.

  3. A prospective, multisite, international validation of the Complex Regional Pain Syndrome Severity Score.

    Science.gov (United States)

    Harden, R Norman; Maihofner, Christian; Abousaad, Elias; Vatine, Jean-Jacques; Kirsling, Amy; Perez, Roberto S G M; Kuroda, Maxine; Brunner, Florian; Stanton-Hicks, Michael; Marinus, Johan; van Hilten, Jacobus J; Mackey, Sean; Birklein, Frank; Schlereth, Tanja; Mailis-Gagnon, Angela; Graciosa, Joe; Connoly, Sara B; Dayanim, David; Massey, Michael; Frank, Hadas; Livshitz, Anatoly; Bruehl, Stephen

    2017-08-01

    Clinical diagnosis of complex regional pain syndrome (CRPS) is a dichotomous (yes/no) categorization, a format necessary for clinical decision making. Such dichotomous diagnostic categories do not convey an individual's subtle gradations in the severity of the condition over time and have poor statistical power when used as an outcome measure in research. This prospective, international, multicenter study slightly modified and further evaluated the validity of the CRPS Severity Score (CSS), a continuous index of CRPS severity. Using a prospective design, medical evaluations were conducted in 156 patients with CRPS to compare changes over time in CSS scores between patients initiating a new treatment program and patients on stable treatment regimens. New vs stable categorizations were supported by greater changes in pain and function in the former. Results indicated that CSS values in the stable CRPS treatment group exhibited much less change over time relative to the new treatment group, with intraclass correlations nearly twice as large in the former. A calculated smallest real difference value revealed that a change in the CSS of ≥4.9 scale points would indicate real differences in CRPS symptomatology (with 95% confidence). Across groups, larger changes in CRPS features on the CSS over time were associated in the expected direction with greater changes in pain intensity, fatigue, social functioning, ability to engage in physical roles, and general well-being. The overall pattern of findings further supports the validity of the CSS as a measure of CRPS severity and suggests it may prove useful in clinical monitoring and outcomes research.

  4. Motor dysfunction of complex regional pain syndrome is related to impaired central processing of proprioceptive information.

    Science.gov (United States)

    Bank, Paulina J M; Peper, C Lieke E; Marinus, Johan; Beek, Peter J; van Hilten, Jacobus J

    2013-11-01

    Our understanding of proprioceptive deficits in complex regional pain syndrome (CRPS) and its potential contribution to impaired motor function is still limited. To gain more insight into these issues, we evaluated accuracy and precision of joint position sense over a range of flexion-extension angles of the wrist of the affected and unaffected sides in 25 chronic CRPS patients and in 50 healthy controls. The results revealed proprioceptive impairment at both the patients' affected and unaffected sides, characterized predominantly by overestimation of wrist extension angles. Precision of the position estimates was more prominently reduced at the affected side. Importantly, group differences in proprioceptive performance were observed not only for tests at identical percentages of each individual's range of wrist motion but also when controls were tested at wrist angles that corresponded to those of the patient's affected side. More severe motor impairment of the affected side was associated with poorer proprioceptive performance. Based on additional sensory tests, variations in proprioceptive performance over the range of wrist angles, and comparisons between active and passive displacements, the disturbances of proprioceptive performance most likely resulted from altered processing of afferent (and not efferent) information and its subsequent interpretation in the context of a distorted "body schema." The present results point at a significant role for impaired central processing of proprioceptive information in the motor dysfunction of CRPS and suggest that therapeutic strategies aimed at identification of proprioceptive impairments and their restoration may promote the recovery of motor function in CRPS patients. Copyright © 2013 American Pain Society. Published by Elsevier Inc. All rights reserved.

  5. Changes in plasma cytokines and their soluble receptors in complex regional pain syndrome.

    Science.gov (United States)

    Alexander, Guillermo M; Peterlin, B Lee; Perreault, Marielle J; Grothusen, John R; Schwartzman, Robert J

    2012-01-01

    Complex Regional Pain Syndrome (CRPS) is a chronic and often disabling pain disorder. There is evidence demonstrating that neurogenic inflammation and activation of the immune system play a significant role in the pathophysiology of CRPS. This study evaluated the plasma levels of cytokines, chemokines, and their soluble receptors in 148 subjects afflicted with CRPS and in 60 gender- and age-matched healthy controls. Significant changes in plasma cytokines, chemokines, and their soluble receptors were found in subjects with CRPS as compared with healthy controls. For most analytes, these changes resulted from a distinct subset of the CRPS subjects. When the plasma data from the CRPS subjects was subjected to cluster analysis, it revealed 2 clusters within the CRPS population. The category identified as most important for cluster separation by the clustering algorithm was TNFα. Cluster 1 consisted of 64% of CRPS subjects and demonstrated analyte values similar to the healthy control individuals. Cluster 2 consisted of 36% of the CRPS subjects and demonstrated significantly elevated levels of most analytes and in addition, it showed that the increased plasma analyte levels in this cluster were correlated with disease duration and severity. The identification of biomarkers that define disease subgroups can be of great value in the design of specific therapies and of great benefit to the design of clinical trials. It may also aid in advancing our understanding of the mechanisms involved in the pathophysiology of CRPS, which may lead to novel treatments for this very severe condition. Copyright © 2012 American Pain Society. Published by Elsevier Inc. All rights reserved.

  6. Rational pain management in complex regional pain syndrome 1 (CRPS 1)--a network meta-analysis.

    Science.gov (United States)

    Wertli, Maria M; Kessels, Alphons G H; Perez, Roberto S G M; Bachmann, Lucas M; Brunner, Florian

    2014-09-01

    Guidelines for complex regional pain syndrome (CRPS) 1 advocate several substance classes to reduce pain and support physical rehabilitation, but guidance about which agent should be prioritized when designing a therapeutic regimen is not provided. Using a network meta-analytic approach, we examined the efficacy of all agent classes investigated in randomized clinical trials of CRPS 1 and provide a rank order of various substances stratified by length of illness duration. In this study a network meta-analysis was conducted. The participants of this study were patients with CRPS 1. Searches in electronic, previous systematic reviews, conference abstracts, book chapters, and the reference lists of relevant articles were performed. Eligible studies were randomized controlled trials comparing at least one analgesic agent with placebo or with another analgesic and reporting efficacy in reducing pain. Summary efficacy stratified by symptom duration and length of follow-up was computed across all substance classes. Two authors independently extracted data. In total, 16 studies were included in the analysis. Bisphosphonates appear to be the treatment of choice in early stages of CRPS 1. The effects of calcitonin surpass that of bisphosphonates and other substances as a short-term medication in more chronic stages of the illness. While most medications showed some efficacy on short-term follow-up, only bisphosphonates, NMDA analogs, and vasodilators showed better long-term pain reduction than placebo. For some drug classes, only a few studies were available and many studies included a small group of patients. Insufficient data were available to analyze efficacy on disability. This network meta-analysis indicates that a rational pharmacological treatment strategy of pain management should consider bisphosphonates in early CRPS 1 and a short-term course of calcitonin in later stages. While most medications showed some efficacy on short-term follow-up, only bisphosphonates

  7. The hypothesis of a syndrome of conservatism on the regional labor market. A draft of the research on regional employees’ and employers’ attitudes towards the development of the professional competence in the region of kujawsko-pomorskie

    Directory of Open Access Journals (Sweden)

    Joanna Szalacha

    2010-12-01

    Full Text Available The article presents some analytical outcomes of the sociological research that took place in June 2009 within companies in kujawsko-pomorski region. The research raised questions about professional training needs of companies and attitudes towards personal abilities, as well as professional development of the company staff. The research was completed by means of an internet poll among the staff of these companies. The article elaborates a hypothesis that there is a specific syndrome of conservatism towards market challenges and different issues of the human capital development. The syndrome concerns both companies and their staff, and can be dangerous for local economic development.

  8. A unique genomic sequence in the Wolf-Hirschhorn syndrome [WHS] region of humans is conserved in the great apes.

    Science.gov (United States)

    Tarzami, S T; Kringstein, A M; Conte, R A; Verma, R S

    1996-10-01

    The Wolf-Hirschhorn syndrome (WHS) is caused by a partial deletion in the short arm of chromosome 4 band 16.3 (4p 16.3). A unique-sequence human DNA probe (39 kb) localized within this region has been used to search for sequence homology in the apes' equivalent chromosome 3 by FISH-technique. The WHS loci are conserved in higher primates at the expected position. Nevertheless, a control probe, which detects alphoid sequences of the pericentromeric region of humans, is diverged in chimpanzee, gorilla, and orangutan. The conservation of WHS loci and divergence of DNA alphoid sequences have further added to the controversy concerning human descent.

  9. Recognizing Wellens’ syndrome, a warning sign of critical proximal LAD artery stenosis and impending anterior myocardial infarction

    Directory of Open Access Journals (Sweden)

    Laura Hollar

    2015-10-01

    Full Text Available Wellens’ syndrome, also known as LAD coronary T-wave syndrome or the ‘widow maker’, is a pre-infarction syndrome with non-classical ischemic ECG changes and unremarkable cardiac biomarkers. This syndrome continues to be a ‘can't miss’ for the clinician as delay in urgent angiography and intervention can result in anterior myocardial infarction, left ventricular dysfunction, arrhythmias, and death. We describe a case followed by a discussion of identification criteria and clinical implications.

  10. [11C]-(R)-PK11195 positron emission tomography in patients with complex regional pain syndrome

    Science.gov (United States)

    Jeon, So Yeon; Seo, Seongho; Lee, Jae Sung; Choi, Soo-Hee; Lee, Do-Hyeong; Jung, Ye-Ha; Song, Man-Kyu; Lee, Kyung-Jun; Kim, Yong Chul; Kwon, Hyun Woo; Im, Hyung-Jun; Lee, Dong Soo; Cheon, Gi Jeong; Kang, Do-Hyung

    2017-01-01

    Abstract Complex regional pain syndrome (CRPS) is characterized by severe and chronic pain, but the pathophysiology of this disease are not clearly understood. The primary aim of our case–control study was to explore neuroinflammation in patients with CRPS using positron emission tomography (PET), with an 18-kDa translocator protein specific radioligand [11C]-(R)-PK11195. [11C]-(R)-PK11195 PET scans were acquired for 11 patients with CRPS (30–55 years) and 12 control subjects (30–52 years). Parametric image of distribution volume ratio (DVR) for each participant was generated by applying a relative equilibrium-based graphical analysis. The DVR of [11C]-(R)-PK11195 in the caudate nucleus (t(21) = −3.209, P = 0.004), putamen (t(21) = −2.492, P = 0.022), nucleus accumbens (t(21) = −2.218, P = 0.040), and thalamus (t(21) = −2.395, P = 0.026) were significantly higher in CRPS patients than in healthy controls. Those of globus pallidus (t(21) = −2.045, P = 0.054) tended to be higher in CRPS patients than in healthy controls. In patients with CRPS, there was a positive correlation between the DVR of [11C]-(R)-PK11195 in the caudate nucleus and the pain score, the visual analog scale (r = 0.661, P = 0.026, R2 = 0.408) and affective subscales of McGill Pain Questionnaire (r = 0.604, P = 0.049, R2 = 0.364). We demonstrated that neuroinflammation of CRPS patients in basal ganglia. Our results suggest that microglial pathology can be an important pathophysiology of CRPS. Association between the level of caudate nucleus and pain severity indicated that neuroinflammation in this region might play a key role. These results may be essential for developing effective medical treatments. PMID:28072713

  11. Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome

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    Francesc Jiménez-Altayó

    2017-11-01

    Full Text Available Marfan syndrome (MFS is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 (Fbn1 allele encoding a missense mutation (Fbn1C1039G/+, the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally

  12. Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome

    Science.gov (United States)

    Jiménez-Altayó, Francesc; Siegert, Anna-Maria; Bonorino, Fabio; Meirelles, Thayna; Barberà, Laura; Dantas, Ana P.; Vila, Elisabet; Egea, Gustavo

    2017-01-01

    Marfan syndrome (MFS) is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 (Fbn1) allele encoding a missense mutation (Fbn1C1039G/+), the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX) inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally, MFS ascending

  13. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012

    DEFF Research Database (Denmark)

    Hjortshøj, Cristel M Sørensen; Jensen, Annette Schophuus; Sørensen, Keld

    2017-01-01

    OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although......-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, p

  14. Huge residual resistivity in the quantum critical region of CeAgSb2

    International Nuclear Information System (INIS)

    Nakashima, Miho; Kirita, Shingo; Asai, Rihito; Kobayashi, Tatsuo C; Okubo, Tomoyuki; Yamada, Mineko; Thamizhavel, Arumugam; Inada, Yoshihiko; Settai, Rikio; Galatanu, Andre; Yamamoto, Etsuji; Ebihara, Takao; Onuki, Yoshichika

    2003-01-01

    We have studied the effect of pressure on the electrical resistivity of a high-quality single crystal CeAgSb 2 which has a small net ferromagnetic moment of 0.4μ B /Ce. The magnetic ordering temperature T ord = 9.7 K decreases with increasing pressure p and disappears at a critical pressure p c ≅ 3.3 GPa. The residual resistivity, which is close to zero up to 3 GPa, increases steeply above 3 GPa, reaching 55μΩ cm at p c . A huge residual resistivity is found to appear when the magnetic order disappears. (letter to the editor)

  15. Gas Bearing Control for Safe Operation in Critical Speed Regions - Experimental Verification

    DEFF Research Database (Denmark)

    Theisen, Lukas R. S.; Niemann, Hans H.; Galeazzi, Roberto

    2015-01-01

    supported by gas bearings to extend their operating range. Using H∞-design methods, active lubrication techniques are proposed to enhance the damping, which in turn reduces the vibrations to a desired safe level. The control design is validated experimentally on a laboratory test rig, and shown to allow...... and deceleration patterns and avoidance of operation near the critical speeds, which is a limiting factor during operation, specially during run-downs. An approach for reducing the vibrations is by feedback controlled lubrication. This paper addresses the challenge of reducing vibrations in rotating machines...

  16. The Usher syndrome in the Lebanese population and further refinement of the USH2A candidate region.

    Science.gov (United States)

    Saouda, M; Mansour, A; Bou Moglabey, Y; El Zir, E; Mustapha, M; Chaib, H; Nehmé, A; Mégarbané, A; Loiselet, J; Petit, C; Slim, R

    1998-08-01

    Usher syndrome (USH) is an autosomal-recessive disease characterized by neurosensory deafness and progressive retinitis pigmentosa. So far, three clinical types of Usher syndrome have been defined, and are caused by defects at more than eight loci. We report the linkage analysis of seven Lebanese families with Usher syndrome, two with type I (USH1) and five with type II (USH2). We demonstrate that one family is linked to the USH1C locus, a rare form of USH1 only reported in the French Acadian population. Linkage analysis of the five USH2 families with recently mapped loci allowed us to reduce the USH2A candidate region to a very small interval flanked by D1S2646/D1S2629 and D1S2827. Furthermore, haplotype comparison between the different families suggests a founder effect for the USH2A mutation among the different Lebanese ethnic groups, while a genetic heterogeneity is noted for Usher syndrome type I.

  17. Structure-guided investigation of lipopolysaccharide O-antigen chain length regulators reveals regions critical for modal length control.

    Science.gov (United States)

    Kalynych, Sergei; Ruan, Xiang; Valvano, Miguel A; Cygler, Miroslaw

    2011-08-01

    The O-antigen component of the lipopolysaccharide (LPS) represents a population of polysaccharide molecules with nonrandom (modal) chain length distribution. The number of the repeat O units in each individual O-antigen polymer depends on the Wzz chain length regulator, an inner membrane protein belonging to the polysaccharide copolymerase (PCP) family. Different Wzz proteins confer vastly different ranges of modal lengths (4 to >100 repeat units), despite having remarkably conserved structural folds. The molecular mechanism responsible for the selective preference for a certain number of O units is unknown. Guided by the three-dimensional structures of PCPs, we constructed a panel of chimeric molecules containing parts of two closely related Wzz proteins from Salmonella enterica and Shigella flexneri which confer different O-antigen chain length distributions. Analysis of the O-antigen length distribution imparted by each chimera revealed the region spanning amino acids 67 to 95 (region 67 to 95), region 200 to 255, and region 269 to 274 as primarily affecting the length distribution. We also showed that there is no synergy between these regions. In particular, region 269 to 274 also influenced chain length distribution mediated by two distantly related PCPs, WzzB and FepE. Furthermore, from the 3 regions uncovered in this study, region 269 to 274 appeared to be critical for the stability of the oligomeric form of Wzz, as determined by cross-linking experiments. Together, our data suggest that chain length determination depends on regions that likely contribute to stabilize a supramolecular complex.

  18. Molecular dissection of a contiguous gene syndrome: Frequent submicroscopic deletions, evolutionarily conserved sequences, and a hypomethylated island in the Miller-Dieker chromosome region

    International Nuclear Information System (INIS)

    Ledbetter, D.H.; Ledbetter, S.A.; vanTuinen, P.

    1989-01-01

    The Miller-Dieker syndrome (MDS), composed of characteristic facial abnormalities and a severe neuronal migration disorder affecting the cerebral cortex, is caused by visible or submicroscopic deletions of chromosome band 17p13. Twelve anonymous DNA markers were tested against a panel of somatic cell hybrids containing 17p deletions from seven MDS patients. All patients, including three with normal karyotypes, are deleted for a variable set of 5-12 markers. Two highly polymorphic VNTR (variable number of tandem repeats) probes, YNZ22 and YNH37, are codeleted in all patients tested and make molecular diagnosis for this disorder feasible. By pulsed-field gel electrophoresis, YNZ22 and YNH37 were shown to be within 30 kilobases (kb) of each other. Cosmid clones containing both VNTR sequences were identified, and restriction mapping showed them to be 100 kb were completely deleted in all patients, providing a minimum estimate of the size of the MDS critical region. A hypomethylated island and evolutionarily conserved sequences were identified within this 100-kb region, indications of the presence of one or more expressed sequences potentially involved in the pathophysiology of this disorder. The conserved sequences were mapped to mouse chromosome 11 by using mouse-rat somatic cell hybrids, extending the remarkable homology between human chromosome 17 and mouse chromosome 11 by 30 centimorgans, into the 17p telomere region

  19. Prevalence of obesity and metabolic syndrome in adult population of selected regions of the Czech Republic. Relation to eating habits and smoking.

    Science.gov (United States)

    Vosátková, M; Ceřovská, J; Zamrazilová, H; Hoskovcová, P; Dvořáková, M; Zamrazil, V

    2012-01-01

    Prevalence of the metabolic syndrome is around 25% in Europe but its occurrence grows in both genders with increasing age and weight. Lifestyle factors may contribute to the risk of developing metabolic syndrome. The objective of this study was to determine the relationship between metabolic syndrome and eating habits as well as length of sleep and smoking. Participants (519 women and 286 men aged 18-65 years) were chosen by random selection and questioned about their eating habits, sleep length and smoking. This information was combined with anthropometric and clinical parameters of metabolic syndrome. The female group was divided into two subgroups depending on climacteric stage (before and after menopause). Metabolic syndrome prevalence does not differ between regions in neither female (29.9%) nor male (32.5%) group. Body mass index ≥25 was detected in 50.4% of all women and 65.7% of men; 23.5% of all women and 21.7% men had body mass index ≥30. In conclusion, metabolic syndrome prevalence was proved to depend on eating habits and family heredity. Positive correlation between the above mentioned factors demonstrated itself in the total sample but not in individual regions. Metabolic syndrome prevalence in Czech adults is comparable with neighbouring countries. No significant interregional differences in metabolic syndrome prevalence within the Czech Republic were detected. In conclusion, relationship between eating habits and metabolic syndrome was confirmed.

  20. Chromosomal microarray testing identifies a 4p terminal region associated with seizures in Wolf–Hirschhorn syndrome

    Science.gov (United States)

    South, Sarah T; Lortz, Amanda; Hensel, Charles H; Sdano, Mallory R; Vanzo, Rena J; Martin, Megan M; Peiffer, Andreas; Lambert, Christophe G; Calhoun, Amy; Carey, John C; Battaglia, Agatino

    2016-01-01

    Background Wolf–Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome involving variable size deletions of the 4p16.3 region. Seizures are frequently, but not always, associated with WHS. We hypothesised that the size and location of the deleted region may correlate with seizure presentation. Methods Using chromosomal microarray analysis, we finely mapped the breakpoints of copy number variants (CNVs) in 48 individuals with WHS. Seizure phenotype data were collected through parent-reported answers to a comprehensive questionnaire and supplemented with available medical records. Results We observed a significant correlation between the presence of an interstitial 4p deletion and lack of a seizure phenotype (Fisher's exact test p=3.59e-6). In our cohort, there were five individuals with interstitial deletions with a distal breakpoint at least 751 kbp proximal to the 4p terminus. Four of these individuals have never had an observable seizure, and the fifth individual had a single febrile seizure at the age of 1.5 years. All other individuals in our cohort whose deletions encompass the terminal 751 kbp region report having seizures typical of WHS. Additional examples from the literature corroborate these observations and further refine the candidate seizure susceptibility region to a region 197 kbp in size, starting 368 kbp from the terminus of chromosome 4. Conclusions We identify a small terminal region of chromosome 4p that represents a seizure susceptibility region. Deletion of this region in the context of WHS is sufficient for seizure occurrence. PMID:26747863

  1. Chromosomal microarray testing identifies a 4p terminal region associated with seizures in Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Ho, Karen S; South, Sarah T; Lortz, Amanda; Hensel, Charles H; Sdano, Mallory R; Vanzo, Rena J; Martin, Megan M; Peiffer, Andreas; Lambert, Christophe G; Calhoun, Amy; Carey, John C; Battaglia, Agatino

    2016-04-01

    Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome involving variable size deletions of the 4p16.3 region. Seizures are frequently, but not always, associated with WHS. We hypothesised that the size and location of the deleted region may correlate with seizure presentation. Using chromosomal microarray analysis, we finely mapped the breakpoints of copy number variants (CNVs) in 48 individuals with WHS. Seizure phenotype data were collected through parent-reported answers to a comprehensive questionnaire and supplemented with available medical records. We observed a significant correlation between the presence of an interstitial 4p deletion and lack of a seizure phenotype (Fisher's exact test p=3.59e-6). In our cohort, there were five individuals with interstitial deletions with a distal breakpoint at least 751 kbp proximal to the 4p terminus. Four of these individuals have never had an observable seizure, and the fifth individual had a single febrile seizure at the age of 1.5 years. All other individuals in our cohort whose deletions encompass the terminal 751 kbp region report having seizures typical of WHS. Additional examples from the literature corroborate these observations and further refine the candidate seizure susceptibility region to a region 197 kbp in size, starting 368 kbp from the terminus of chromosome 4. We identify a small terminal region of chromosome 4p that represents a seizure susceptibility region. Deletion of this region in the context of WHS is sufficient for seizure occurrence. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  2. [Application of Chinese Medical Syndrome Scores in Effectiveness Evaluation: a Critical Appraisal of 240 Randomized Controlled Trials].

    Science.gov (United States)

    Luo, Hui; Liao, Xing; Wang, Qian

    2015-10-01

    To explore the role of traditional Chinese medicine (TCM) syndrome scores in effectiveness evaluation of clinical studies. Randomized controlled trials (RCTs) of TCM published in five journals in 2013 were retrieved, including Journal of Traditional Chinese Medicine (JTCM), Chinese Journal of Integrated Traditional and Western Medicine (CJITWM), Chinese Journal of Integrative Medicine (CJIM), Evidence-Based Complementary and Alternative Medicine (ECAM), and American Journal of Chinese Medicine (AJCM). The details of TCM syndrome scores and other relevant factors reported in articles were extracted and analyzed. Descriptive statistics and Chi-square test were used to describe general features of inclusive studies, ratios of reports on CM syndrome scores in each journal, formulated evidence, adopted evaluation rules, important degrees. The difference in the application rate of CM syndrome scores were compared in various diseases, diseases with or without CM syndrome typing, places where clinical studies were implemented, and different journals. A total of 240 RCTs were included, involving 178 published in Chinese version and 62 in English version. CM syndrome scores were used for effectiveness evaluation in 27.1% (65/240) of RCTs, of which, the highest application percentage was 35.3% (18/51) in RCTs published in CJITWM, and the lowest was 0 (0/7) in RCTs published in AJCM. There were 17 methods for grading TCM syndrome scores, of which Guideline for Clinical Research of New Chinese Herbal Medicine was most commonly used. Detailed grading standards for CM syndrome scores were reported in 46 RCTs, and CM syndrome scores were taken as primary or secondary outcomes in 6 RCTs. When describing the percentages of RCTs adopting TCM syndrome score by diseases classification, the lowest was 9.5% (2/21) in mental and behavioral disorders, and the highest was 39.1% (9/23) in endocrine, nutritional, and metabolic diseases. RCTs with TCM syndrome differentiation had a higher

  3. A critical readjustment : Analyzing the regional employment composition in Norway during times of change

    OpenAIRE

    Gramstad, Emma Hartland; Wærness, Katrine Willumsen

    2016-01-01

    The dramatic fall in the oil price, which started in June 2014, introduced a national debate concerning the future of the petroleum sector in Norway. This thesis examines the sectoral employment composition in Norway, and expands the country-level analysis by looking at regional differences. The main focus is on Rogaland, which is clearly dependent on the petroleum sector. Rogaland’s development is compared to three counties that are presumed to be less petroleum dependent, nam...

  4. Identification of conserved regions and residues within Hedgehog acyltransferase critical for palmitoylation of Sonic Hedgehog.

    Directory of Open Access Journals (Sweden)

    John A Buglino

    2010-06-01

    Full Text Available Sonic hedgehog (Shh is a palmitoylated protein that plays key roles in mammalian development and human cancers. Palmitoylation of Shh is required for effective long and short range Shh-mediated signaling. Attachment of palmitate to Shh is catalyzed by Hedgehog acyltransferase (Hhat, a member of the membrane bound O-acyl transferase (MBOAT family of multipass membrane proteins. The extremely hydrophobic composition of MBOAT proteins has limited their biochemical characterization. Except for mutagenesis of two conserved residues, there has been no structure-function analysis of Hhat, and the regions of the protein required for Shh palmitoylation are unknown.Here we undertake a systematic approach to identify residues within Hhat that are required for protein stability and/or enzymatic activity. We also identify a second, novel MBOAT homology region (residues 196-234 that is required for Hhat activity. In total, ten deletion mutants and eleven point mutants were generated and analyzed. Truncations at the N- and C-termini of Hhat yielded inactive proteins with reduced stability. Four Hhat mutants with deletions within predicted loop regions and five point mutants retained stability but lost palmitoylation activity. We purified two point mutants, W378A and H379A, with defective Hhat activity. Kinetic analyses revealed alterations in apparent K(m and V(max for Shh and/or palmitoyl CoA, changes that likely explain the catalytic defects observed for these mutants.This study has pinpointed specific regions and multiple residues that regulate Hhat stability and catalysis. Our findings should be applicable to other MBOAT proteins that mediate lipid modification of Wnt proteins and ghrelin, and should serve as a model for understanding how secreted morphogens are modified by palmitoyl acyltransferases.

  5. Probing the critical behavior in the evolution of GDR width at very low temperatures in A∼100 mass region

    Energy Technology Data Exchange (ETDEWEB)

    Dey, Balaram; Mondal, Debasish; Pandit, Deepak; Mukhopadhyay, S.; Pal, Surajit [Variable Energy Cyclotron Centre, 1/AF-Bidhannagar, Kolkata 700064 (India); Bhattacharya, Srijit [Department of Physics, Barasat Govt. College, Barasat, N 24 Pgs, Kolkata 700124 (India); De, A. [Department of Physics, Raniganj Girls' College, Raniganj 713358 (India); Banerjee, K. [Variable Energy Cyclotron Centre, 1/AF-Bidhannagar, Kolkata 700064 (India); Dinh Dang, N. [Theoretical Nuclear Physics Laboratory, RIKEN Nishina Center for Accelerator-Based Science, RIKEN, 2-1 Hirosawa, Wako city, Saitama 351-0198 (Japan); Quang Hung, N. [School of Engineering, Tan Tao University, Tan Tao University Avenue, Tan Duc Ecity, Duc Hoa, Long An Province (Viet Nam); Banerjee, S.R., E-mail: srb@vecc.gov.in [Variable Energy Cyclotron Centre, 1/AF-Bidhannagar, Kolkata 700064 (India)

    2014-04-04

    The influence of giant dipole resonance (GDR) induced quadrupole moment on GDR width at low temperatures is investigated experimentally by measuring GDR width systematically in the unexplored temperature range T=0.8–1.5 MeV, for the first time, in A∼100 mass region. The measured GDR widths, using alpha induced fusion reaction, for {sup 97}Tc confirm that the GDR width remains constant at the ground state value up to a critical temperature and increases sharply thereafter with increase in T. The data have been compared with the adiabatic Thermal Shape Fluctuation Model (TSFM), phenomenological Critical Temperature Fluctuation Model (CTFM) and microscopic Phonon Damping Model (PDM). Interestingly, CTFM and PDM give similar results and agree with the data, whereas the TSFM differs significantly even after incorporating the shell effects.

  6. Probing the critical behavior in the evolution of GDR width at very low temperatures in A∼100 mass region

    International Nuclear Information System (INIS)

    Dey, Balaram; Mondal, Debasish; Pandit, Deepak; Mukhopadhyay, S.; Pal, Surajit; Bhattacharya, Srijit; De, A.; Banerjee, K.; Dinh Dang, N.; Quang Hung, N.; Banerjee, S.R.

    2014-01-01

    The influence of giant dipole resonance (GDR) induced quadrupole moment on GDR width at low temperatures is investigated experimentally by measuring GDR width systematically in the unexplored temperature range T=0.8–1.5 MeV, for the first time, in A∼100 mass region. The measured GDR widths, using alpha induced fusion reaction, for 97 Tc confirm that the GDR width remains constant at the ground state value up to a critical temperature and increases sharply thereafter with increase in T. The data have been compared with the adiabatic Thermal Shape Fluctuation Model (TSFM), phenomenological Critical Temperature Fluctuation Model (CTFM) and microscopic Phonon Damping Model (PDM). Interestingly, CTFM and PDM give similar results and agree with the data, whereas the TSFM differs significantly even after incorporating the shell effects.

  7. Critical strain region evaluation of self-assembled semiconductor quantum dots

    Energy Technology Data Exchange (ETDEWEB)

    Sales, D L [Departamento de Ciencia de los Materiales e I. M. y Q. I., Universidad de Cadiz, Puerto Real, Cadiz (Spain); Pizarro, J [Departamento de Lenguajes y Sistemas Informaticos, Universidad de Cadiz, Puerto Real, Cadiz (Spain); Galindo, P L [Departamento de Lenguajes y Sistemas Informaticos, Universidad de Cadiz, Puerto Real, Cadiz (Spain); Garcia, R [Departamento de Ciencia de los Materiales e I. M. y Q. I., Universidad de Cadiz, Puerto Real, Cadiz (Spain); Trevisi, G [CNR-IMEM Institute, Parco delle Scienze 37a, 43100, Parma (Italy); Frigeri, P [CNR-IMEM Institute, Parco delle Scienze 37a, 43100, Parma (Italy); Nasi, L [CNR-IMEM Institute, Parco delle Scienze 37a, 43100, Parma (Italy); Franchi, S [CNR-IMEM Institute, Parco delle Scienze 37a, 43100, Parma (Italy); Molina, S I [Departamento de Ciencia de los Materiales e I. M. y Q. I., Universidad de Cadiz, Puerto Real, Cadiz (Spain)

    2007-11-28

    A novel peak finding method to map the strain from high resolution transmission electron micrographs, known as the Peak Pairs method, has been applied to In(Ga)As/AlGaAs quantum dot (QD) samples, which present stacking faults emerging from the QD edges. Moreover, strain distribution has been simulated by the finite element method applying the elastic theory on a 3D QD model. The agreement existing between determined and simulated strain values reveals that these techniques are consistent enough to qualitatively characterize the strain distribution of nanostructured materials. The correct application of both methods allows the localization of critical strain zones in semiconductor QDs, predicting the nucleation of defects, and being a very useful tool for the design of semiconductor devices.

  8. Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1).

    Science.gov (United States)

    Fischer, Sigrid G L; Collins, Susan; Boogaard, Sabine; Loer, Stephan A; Zuurmond, Wouter W A; Perez, Roberto S G M

    2013-09-01

    To assess the effects of intravenous administration of magnesium on complex regional pain syndrome type 1 (CRPS-1), a randomized double-blind placebo-controlled trial was performed. Fifty-six patients with CRPS-1 (International Association for the Study of Pain Orlando criteria) received MgSO(4) 70 mg/kg or placebo (NaCl 0.9%) in 4 hours over 5 consecutive days. Pain (BOX-11 and McGill), the level of impairment (Impairment level Sum Score [ISS]), functional limitations (Radboud Skills Questionnaire, Walking Skills Questionnaire/questionnaire rising and sitting down), participation (Impact on Participation and Autonomy [IPA]), and quality of life (Short Form-36, EuroQol, IPA) were evaluated at baseline and at 1, 3, 6, and 12 weeks. No significant differences were found between MgSO(4) and placebo on the BOX-11 and ISS at different time points during the trial on intention-to-treat and per-protocol analysis. A significant improvement on the BOX-11 was found after the first week of the trial in both groups (mean 0.7; standard deviation 1.1). For the MgSO(4) group, a clinically relevant and statistically significant improvement on the ISS at 1 week (median 5, interquartile range [IQR] -1 to 8) and a significant improvement on the McGill up to 6 weeks (median 2 words, IQR 0-4.5) were found compared with baseline, which were not found in the placebo group. Significant improvement in perceived job participation was found for the MgSO(4) group at 12 weeks (median improvement 1.44-1.17; P = 0.01). ISS improved significantly more in patients with a low Hospital Anxiety and Depression Scale (HADS) score (≤10) in the MgSO(4) group (mean 4.4 vs mean -3.1; P = 0.02). Administration of the physiological competitive N-methyl-D-aspartate receptor antagonist magnesium in chronic CRPS provides insufficient benefit over placebo. Future research should focus on patients with acute CRPS and early signs and symptoms of central sensitization. Wiley Periodicals, Inc.

  9. Prevalence and Cost Analysis of Complex Regional Pain Syndrome (CRPS): A Role for Neuromodulation.

    Science.gov (United States)

    Elsamadicy, Aladine A; Yang, Siyun; Sergesketter, Amanda R; Ashraf, Bilal; Charalambous, Lefko; Kemeny, Hanna; Ejikeme, Tiffany; Ren, Xinru; Pagadala, Promila; Parente, Beth; Xie, Jichun; Lad, Shivanand P

    2017-09-29

    The diagnosis and treatment of complex regional pain syndrome (CRPS) is challenging and there is a paucity of data describing its overall cost burden and quantifying its impact on the US healthcare system. The aim of this study was to assess the prevalence and healthcare utilization costs associated with CRPS. A retrospective longitudinal study was performed using the Truven MarketScan® database to identify patients with a new indexed diagnosis of CRPS (Type I, II, or both) from 2001 to 2012. We collected total, outpatient, and pain prescription costs three years prior to CRPS diagnosis (baseline), at year of CRPS diagnosis, and eight-year post-CRPS diagnosis. A longitudinal multivariate analysis was used to model the estimated total and pain prescription cost ratios comparing patients diagnosed before and after CRPS. We included 35,316 patients with a newly indexed diagnosis of CRPS (Type I: n = 18,703, Type II: n = 14,599, Unspecified: n = 2014). Baseline characteristics were similar between the CRPS cohorts. Compared to two- and three-year baseline costs, one-year prior to diagnosis for all CRPS patients yielded the highest interquartile median [IQR] costs: total costs $7904[$3469, $16,084]; outpatient costs $6706[$3119, $12,715]; and pain prescription costs $1862[$147, $7649]. At the year of CRPS diagnosis, the median [IQR] costs were significantly higher than baseline costs: total costs $8508[$3943, $16,666]; outpatient costs $7251[$3527, $13,568]; and pain prescription costs $2077[$140, $8856]. Over the eight-year period after CRPS diagnosis, costs between all the years were similar, ranging from the highest (one-year) to lowest (seven-years), $4845 to $3888. The median total cumulative cost 8-years after CRPS diagnosis was $43,026 and $12,037 for pain prescription costs. [Correction added on 06 November 2017 after first online publication: the preceding sentence has been updated to demonstrate the median cumulative cost in replacement of the

  10. Primary drug resistance in a region with high burden of tuberculosis. A critical problem.

    Science.gov (United States)

    Villa-Rosas, Cecilia; Laniado-Laborín, Rafael; Oceguera-Palao, Lorena

    2015-01-01

    To determine rates of drug resistance in new cases of pulmonary tuberculosis in a region with a high burden of the disease. New case suspects were referred for drug susceptibility testing. 28.9% of new cases were resistant to at least one first line drug; 3.9% had a multidrug-resistant strain, 15.6% a monoresistant strain and 9.4% a polyresistant strain. Our rate of drug resistant tuberculosis in new cases is very high; this has important clinical implications, since even monoresistance can have a negative impact on the outcome of new cases treated empirically with a six month regimen.

  11. Complex Regional Pain Syndrome Revived by Epileptic Seizure Then Disappeared Soon during Treatment with Regional Intravenous Nerve Blockade: A Case Report

    Directory of Open Access Journals (Sweden)

    Masahiko Sumitani

    2011-01-01

    Full Text Available We present a case of complex regional pain syndrome (CRPS, in which symptoms, including burning pain and severe allodynia, were alleviated by using a regional intravenous nerve blockade (Bier block combined with physiotherapy, but reappeared following an epileptic seizure. Symptoms disappeared again following control of epileptic discharges, as revealed by single-photon emission computed tomography (SPECT and electroencephalography (EEG results. Although systemic toxicity of a local anesthetic applied by Bier block was suspected as a cause of the first seizure, the patient did not present any other toxic symptoms, and seizures repeatedly occurred after Bier block cessation; the patient was then diagnosed as having temporal symptomatic epilepsy. This case suggests that symptoms of CRPS may be sustained by abnormal brain conditions, and our findings contribute to the understanding of how the central nervous system participates in maintaining pain and allodynia associated with CRPS.

  12. Phase analysis of regional and global ventricular contraction patterns in Wolff-Parkinson-White syndrome

    International Nuclear Information System (INIS)

    Konishi, Tokuji; Koyama, Takao; Ichikawa, Takehiko

    1989-01-01

    Multigated blood pool scintigraphy was performed in 20 normal subjects and 39 patients with various intraventricular conduction abnormalities, including 25 patients with Wolff-Parkinson-White (WPW) syndrome. Cardiac imaging was performed in the modified left anterior oblique, right anterior oblique, and left lateral projections. In WPW syndrome, early contraction sites which were not seen in normal subjects were detected at the ventricular base in phase images. These anomalous early contraction sites disappeared after successful suppression of conduction through an accessory pathway by intravenous procainamide. These sites are believed to correspond to the location of the bundle of Kent and were consistent with the electrocardiographic findings. Phase mapping is a suitable noninvasive method to locate the position of the bundle of Kent and evaluate the ventricular contraction pattern in WPW syndrome and other intraventricular conduction abnormalities. (author)

  13. The rostral medulla of bullfrog tadpoles contains critical lung rhythmogenic and chemosensitive regions across metamorphosis.

    Science.gov (United States)

    Reed, Mitchell D; Iceman, Kimberly E; Harris, Michael B; Taylor, Barbara E

    2018-06-08

    The development of amphibian breathing provides insight into vertebrate respiratory control mechanisms. Neural oscillators in the rostral and caudal medulla drive ventilation in amphibians, and previous reports describe ventilatory oscillators and CO 2 sensitive regions arise during different stages of amphibian metamorphosis. However, inconsistent findings have been enigmatic, and make comparisons to potential mammalian counterparts challenging. In the current study we assessed amphibian central CO 2 responsiveness and respiratory rhythm generation during two different developmental stages. Whole-nerve recordings of respiratory burst activity in cranial and spinal nerves were made from intact or transected brainstems isolated from tadpoles during early or late stages of metamorphosis. Brainstems were transected at the level of the trigeminal nerve, removing rostral structures including the nucleus isthmi, midbrain, and locus coeruleus, or transected at the level of the glossopharyngeal nerve, removing the putative buccal oscillator and caudal medulla. Removal of caudal structures stimulated the frequency of lung ventilatory bursts and revealed a hypercapnic response in normally unresponsive preparations derived from early stage tadpoles. In preparations derived from late stage tadpoles, removal of rostral or caudal structures reduced lung burst frequency, while CO 2 responsiveness was retained. Our results illustrate that structures within the rostral medulla are capable of sensing CO 2 throughout metamorphic development. Similarly, the region controlling lung ventilation appears to be contained in the rostral medulla throughout metamorphosis. This work offers insight into the consistency of rhythmic respiratory and chemosensitive capacities during metamorphosis. Copyright © 2018. Published by Elsevier Inc.

  14. Primary drug resistance in a region with high burden of tuberculosis. A critical problem

    Directory of Open Access Journals (Sweden)

    Cecilia Villa-Rosas

    2015-03-01

    Full Text Available Objective. To determine rates of drug resistance in new cases of pulmonary tuberculosis in a region with a high burden of the disease. Materials and methods. New case suspects were referred for drug susceptibility testing. Results. 28.9% of new cases were resistant to at least one first line drug; 3.9% had a multidrug-resistant strain, 15.6% a monoresistant strain and 9.4% a polyresistant strain. Conclusion. Our rate of drug resistant tuberculosis in new cases is very high; this has important clinical implications, since even monoresistance can have a negative impact on the outcome of new cases treated empirically with a six month regimen.

  15. Aging rather than aneuploidy affects monoamine neurotransmitters in brain regions of Down syndrome mouse models

    NARCIS (Netherlands)

    Dekker, Alain D; Vermeiren, Yannick; Albac, Christelle; Lana-Elola, Eva; Watson-Scales, Sheona; Gibbins, Dorota; Aerts, Tony; Van Dam, Debby; Fisher, Elizabeth M C; Tybulewicz, Victor L J; Potier, Marie-Claude; De Deyn, Peter P

    Altered concentrations of monoamine neurotransmitters and metabolites have been repeatedly found in people with Down syndrome (DS, trisomy 21). Because of the limited availability of human post-mortem tissue, DS mouse models are of great interest to study these changes and the underlying

  16. Geochemical characterization of critical dust source regions in the American West

    Science.gov (United States)

    Aarons, Sarah M.; Blakowski, Molly A.; Aciego, Sarah M.; Stevenson, Emily I.; Sims, Kenneth W. W.; Scott, Sean R.; Aarons, Charles

    2017-10-01

    The generation, transport, and deposition of mineral dust are detectable in paleoclimate records from land, ocean, and ice, providing valuable insight into earth surface conditions and cycles on a range of timescales. Dust deposited in marine and terrestrial ecosystems can provide critical nutrients to nutrient-limited ecosystems, and variations in dust provenance can indicate changes in dust production, sources and transport pathways as a function of climate variability and land use change. Thus, temporal changes in locations of dust source areas and transport pathways have implications for understanding interactions between mineral dust, global climate, and biogeochemical cycles. This work characterizes dust from areas in the American West known for dust events and/or affected by increasing human settlement and livestock grazing during the last 150 years. Dust generation and uplift from these dust source areas depends on climate and land use practices, and the relative contribution of dust has likely changed since the expansion of industrialization and agriculture into the western United States. We present elemental and isotopic analysis of 28 potential dust source area samples analyzed using Thermal Ionization Mass Spectrometry (TIMS) for 87Sr/86Sr and 143Nd/144Nd composition and Multi-Collector Inductively Coupled Plasma Mass Spectrometer (MC-ICPMS) for 176Hf/177Hf composition, and ICPMS for major and trace element concentrations. We find significant variability in the Sr, Nd, and Hf isotope compositions of potential source areas of dust throughout western North America, ranging from 87Sr/86Sr = 0.703699 to 0.740236, εNd = -26.6 to 2.4, and εHf = -21.7 to -0.1. We also report differences in the trace metal and phosphorus concentrations in the geologic provinces sampled. This research provides an important resource for the geochemical tracing of dust sources and sinks in western North America, and will aid in modeling the biogeochemical impacts of increased

  17. Test of the critical point symmetry X(5) in the mass region A=180

    Energy Technology Data Exchange (ETDEWEB)

    Melon, B.; Dewald, A.; Moeller, O.; Pissulla, T.; Fransen, C.; Jolie, J.; Linnemann, A.; Zell, K.O. [IKP, Univ. zu Koeln (Germany); Petkov, P. [Bulg. Acad. of Sciences, Inst. for Nucl. Res. and Nucl. Ener., Sofia (Bulgaria); Tonev, D.; Angelis, G. De; Bazzacco, D. [INFN, LN di Legnaro (Italy); Ur, C.; Menegazzo, R.; Farnea, E. [Dip. di Fisica, Univ.' and INFN Sez. Padova (Italy)

    2007-07-01

    Recently, the first examples of X(5) like nuclei in the mass region A=180 have been identified, {sup 176,178}Os. We report on further results of a lifetime measurement performed at the LN Legnaro using the Koeln coincidence plunger device and the GASP spectrometer. Excited states were populated via the reaction {sup 152}Sm({sup 29}Si,5n){sup 176}Os at E({sup 29}Si) = 145 MeV. Special effort has been made to reduce the experimental uncertainty of the B(E2, 2{sub 1}{sup +} {yields} 0{sub 1}{sup +}) value in {sup 178}Os, used for transition strength normalizations in {sup 178}Os necessary for the comparison with theoretical models. A second experiment was performed to measure the lifetime of the first excited 2{sup +} state in {sup 178}Os with the Koeln coincidence plunger device at the FN Tandem accelerator of the University of Cologne using THE {sup 166}Er({sup 16}O,4n){sup 178}Os reaction at E({sup 16}O) = 80 MeV. The resulting lifetime has been compared to the value obtained using the delayed coincidence method. (orig.)

  18. Critical role of dendritic cells in T cell retention in the interfollicular region of Peyer's patches.

    Science.gov (United States)

    Obata, Takashi; Shibata, Naoko; Goto, Yoshiyuki; Ishikawa, Izumi; Sato, Shintaro; Kunisawa, Jun; Kiyono, Hiroshi

    2013-07-15

    Peyer's patches (PPs) simultaneously initiate active and quiescent immune responses in the gut. The immunological function is achieved by the rigid regulation of cell distribution and trafficking, but how the cell distribution is maintained remains to be elucidated. In this study, we show that binding of stromal cell-derived lymphoid chemokines to conventional dendritic cells (cDCs) is essential for the retention of naive CD4(+) T cells in the interfollicular region (IFR) of PPs. Transitory depletion of CD11c(high) cDCs in mice rapidly impaired the IFR structure in the PPs without affecting B cell follicles or germinal centers, lymphoid chemokine production from stromal cells, or the immigration of naive T cells into the IFRs of PPs. The cDC-orchestrated retention of naive T cells was mediated by heparinase-sensitive molecules that were expressed on cDCs and bound the lymphoid chemokine CCL21 produced from stromal cells. These data collectively reveal that interactions among cDCs, stromal cells, and naive T cells are necessary for the formation of IFRs in the PPs.

  19. Photoacoustic microscopy of complex regional pain syndrome type I (CRPS-1) after stellate ganglion blocks in vivo

    Science.gov (United States)

    Zhou, Yong; Yi, Xiaobin; Xing, Wenxin; Hu, Song; Maslov, Konstantin I.; Wang, Lihong V.

    2015-03-01

    We used photoacoustic microscopy (PAM) to assist diagnoses and monitor the progress and treatment outcome of complex regional pain syndrome type 1 (CRPS-1). Blood vasculature and oxygen saturation (sO2) were imaged by PAM in eight adult patients with CRPS-1. Patients' hands and cuticles were imaged both before and after stellate ganglion block (SGB) for comparison. For all patients, both the vascular structure and sO2 could be assessed by PAM. In addition, more vessels and stronger signals were observed after SGB.

  20. The Sertoli Cell Only Syndrome and Glaucoma in a Sex - Determining Region Y (SRY) Positive XX Infertile Male.

    Science.gov (United States)

    Jain, Manish; V, Veeramohan; Chaudhary, Isha; Halder, Ashutosh

    2013-07-01

    The XX male syndrome is a rare genetic disorder. The phenotype is variable; it ranges from a severe impairment of the external genitalia to a normal male phenotype with infertility. It generally results from an unequal crossing over between the short arms of the sex chromosomes (X and Y). We are reporting a case of a 38-year-old man who presented with infertility and the features of hypogonadism and glaucoma. The examinations revealed normal external male genitalia, soft small testes, gynaecomastia and glaucoma. The semen analysis showed azoospermia. The serum gonadotropins were high, with low Anti Mullerian Hormone (AMH) and Inhibin B levels. The chromosomal analysis demonstrated a 46, XX karyotype. Fluorescent In-Situ Hybridization (FISH) and Polymerase Chain Reaction (PCR) revealed the presence of a Sex-determining Region Y (SRY). Testicular Fine Needle Aspiration Cytology (FNAC) revealed the Sertoli Cell Only Syndrome (SCOS). The presence of only Sertoli Cells in the testes, with glaucoma in the XX male syndrome, to our knowledge, has not been reported in the literature.

  1. Priority persistent contaminants in people dwelling in critical areas of Campania Region, Italy (SEBIOREC biomonitoring study)

    International Nuclear Information System (INIS)

    De Felip, Elena; Bianchi, Fabrizio; Bove, Crescenzo; Cori, Liliana; D'Argenzio, Angelo; D'Orsi, Giancarlo; Fusco, Mario; Miniero, Roberto; Ortolani, Rosanna; Palombino, Raffaele; Parlato, Antonino; Pelliccia, Maria Grazia; Peluso, Filomena; Piscopo, Giovanni; Pizzuti, Renato; Porpora, Maria Grazia; Protano, Domenico; Senofonte, Oreste

    2014-01-01

    To investigate if protracted living in degraded environments of the Caserta and Naples provinces (Campania Region, Italy) had an impact on exposure of local people, highly toxic persistent contaminants were measured in blood, blood serum, and human milk of a large number of healthy donors. Sampling was carried out from 2008 to 2009. Blood was collected from over 850 20–64-year old donors; by pooling, 84 blood and 84 serum samples were obtained. Milk was donated by 52 mothers: specimens were pooled into six samples. Polychlorodibenzodioxins (PCDDs), polychlorodibenzofurans (PCDFs), and polychlorobiphenyls (PCBs, dioxin-like (DL) and non-dioxin-like (Σ 6 PCBs)), arsenic (As), cadmium (Cd), mercury (Hg), and lead (Pb) were measured in serum (organic biomarkers) and blood (metals); these chemicals and polybromobiphenyl ethers (Σ 9 PBDEs) were analyzed in milk. PCDD + PCDF, DL-PCB, TEQ TOT , and Σ 6 PCB concentration ranges (medians) in serum were 6.26–23.1 (12.4), 3.42–31.7 (11.5), 10.0–52.8 (23.9) pgTEQ 97 /g fat, and 55.5–647 (219) ng/g fat, respectively, while in milk concentration ranges were 5.99–8.77, 4.02–6.15, 10.0–14.2 pgTEQ 97 /g fat, and 48.7–74.2 ng/g fat. Likewise, As, Cd, Hg, and Pb findings in blood spanned 2.34–13.4 (5.83), 0.180–0.930 (0.475), 1.09–7.60 (2.60), 10.2–55.9 (28.8) μg/L, respectively; only Pb could be measured in milk (2.78–5.99 μg/L). Σ 9 PBDE levels in milk samples were 0.965–6.05 ng/g fat. Biomarkers' concentrations were found to be compatible with their current values in European countries and in Italy, and consistent with an exposure primarily determined by consumption of commercial food from the large distribution system. Based on relatively higher biomarker values within the hematic biomonitoring database, the following municipalities were flagged as possibly deserving attention for health-oriented interventions: Brusciano and Caivano (As), Giugliano (Hg), Pianura (PCDDs + PCDFs), and Qualiano

  2. Priority persistent contaminants in people dwelling in critical areas of Campania Region, Italy (SEBIOREC biomonitoring study)

    Energy Technology Data Exchange (ETDEWEB)

    De Felip, Elena, E-mail: defelip@iss.it [Istituto Superiore di Sanità, Dipartimento Ambiente e connessa Prevenzione Primaria, Rome (Italy); Bianchi, Fabrizio [Consiglio Nazionale delle Ricerche, Istituto di Fisiologia Clinica, Pisa and Rome (Italy); Bove, Crescenzo [ASL CE, Servizio di Epidemiologia e Prevenzione, Caserta (Italy); Cori, Liliana [Consiglio Nazionale delle Ricerche, Istituto di Fisiologia Clinica, Pisa and Rome (Italy); D' Argenzio, Angelo [ASL CE, Servizio di Epidemiologia e Prevenzione, Caserta (Italy); D' Orsi, Giancarlo [ASL NA2 Nord, Servizio di Epidemiologia e Prevenzione, Naples (Italy); Fusco, Mario [Registro Tumori della Regione Campania, ASL NA3 Sud, Naples (Italy); Miniero, Roberto [Istituto Superiore di Sanità, Dipartimento Ambiente e connessa Prevenzione Primaria, Rome (Italy); Ortolani, Rosanna [ASL NA1 Centro, Servizio di Epidemiologia e Prevenzione, Naples (Italy); Palombino, Raffaele [ASL NA3 Sud, Servizio di Epidemiologia e Prevenzione, Distretto Sanitario 69, Naples (Italy); Parlato, Antonino; Pelliccia, Maria Grazia; Peluso, Filomena [ASL NA2 Nord, Servizio di Epidemiologia e Prevenzione, Naples (Italy); Piscopo, Giovanni [ASL NA3 Sud, Servizio di Epidemiologia e Prevenzione, Distretto Sanitario 69, Naples (Italy); Pizzuti, Renato [Regione Campania, Assessorato alla Sanità, Osservatorio Epidemiologico, Naples (Italy); Porpora, Maria Grazia [Dipartimento di Ginecologia e Ostetricia, Dipartimento di Scienze Ginecologiche, Perinatologia, e Puericultura, Policlinico Umberto I, Università “Sapienza”, Rome (Italy); Protano, Domenico [ASL CE, Servizio di Epidemiologia e Prevenzione, Caserta (Italy); Senofonte, Oreste [Istituto Superiore di Sanità, Dipartimento Ambiente e connessa Prevenzione Primaria, Rome (Italy); and others

    2014-07-01

    To investigate if protracted living in degraded environments of the Caserta and Naples provinces (Campania Region, Italy) had an impact on exposure of local people, highly toxic persistent contaminants were measured in blood, blood serum, and human milk of a large number of healthy donors. Sampling was carried out from 2008 to 2009. Blood was collected from over 850 20–64-year old donors; by pooling, 84 blood and 84 serum samples were obtained. Milk was donated by 52 mothers: specimens were pooled into six samples. Polychlorodibenzodioxins (PCDDs), polychlorodibenzofurans (PCDFs), and polychlorobiphenyls (PCBs, dioxin-like (DL) and non-dioxin-like (Σ{sub 6}PCBs)), arsenic (As), cadmium (Cd), mercury (Hg), and lead (Pb) were measured in serum (organic biomarkers) and blood (metals); these chemicals and polybromobiphenyl ethers (Σ{sub 9}PBDEs) were analyzed in milk. PCDD + PCDF, DL-PCB, TEQ{sub TOT}, and Σ{sub 6}PCB concentration ranges (medians) in serum were 6.26–23.1 (12.4), 3.42–31.7 (11.5), 10.0–52.8 (23.9) pgTEQ{sub 97}/g fat, and 55.5–647 (219) ng/g fat, respectively, while in milk concentration ranges were 5.99–8.77, 4.02–6.15, 10.0–14.2 pgTEQ{sub 97}/g fat, and 48.7–74.2 ng/g fat. Likewise, As, Cd, Hg, and Pb findings in blood spanned 2.34–13.4 (5.83), 0.180–0.930 (0.475), 1.09–7.60 (2.60), 10.2–55.9 (28.8) μg/L, respectively; only Pb could be measured in milk (2.78–5.99 μg/L). Σ{sub 9}PBDE levels in milk samples were 0.965–6.05 ng/g fat. Biomarkers' concentrations were found to be compatible with their current values in European countries and in Italy, and consistent with an exposure primarily determined by consumption of commercial food from the large distribution system. Based on relatively higher biomarker values within the hematic biomonitoring database, the following municipalities were flagged as possibly deserving attention for health-oriented interventions: Brusciano and Caivano (As), Giugliano (Hg), Pianura

  3. Pendred syndrome (goitre and sensorineural hearing loss) maps to chromosome 7 in the region containing the nonsyndromic deafness gene DFNB4.

    Science.gov (United States)

    Coyle, B; Coffey, R; Armour, J A; Gausden, E; Hochberg, Z; Grossman, A; Britton, K; Pembrey, M; Reardon, W; Trembath, R

    1996-04-01

    Inherited causes account for about 50% of individuals presenting with childhood (prelingual) hearing loss, of which 70% are due to mutation in numerous single genes which impair auditory function alone (non-syndromic). The remainder are associated with other developmental anomalies termed syndromic deafness. Genes responsible for syndromic forms of hearing loss include the COL4A5 gene in Alport syndrome and the PAX3 and MITF genes in Waardenburg syndrome. Pendred syndrome is an autosomal recessive disorder associated with developmental abnormalities of the cochlea, sensorineural hearing loss and diffuse thyroid enlargement (goitre). Pendred syndrome is the most common syndromal form of deafness, yet the primary defect remains unknown. We have established a panel of 12 families with two or more affected individuals and used them to search for the location of the Pendred gene by linkage analysis. We excluded localization to four previously mapped nonsyndromic deafness loci but obtained conclusive evidence for linkage of the Pendred syndrome gene to microsatellite markers on chromosome 7q31 (D7S495 Zmax 7.32, Qmax = 0). This region contains a gene, DFNBL, for autosomal recessive non-syndromic sensorineural hearing loss. Multipoint analysis indicates that DFNB4 and Pendred syndrome co-localize to the same 5.5 centiMorgan (cM) interval flanked by D7S501 and D7S523. These data raise the possibility that Pendred syndrome is either allelic with DFNB4 or may represent an inherited contiguous gene disorder, not clinically manifest in the heterozygote.

  4. Identification of critical regions in human SAMHD1 required for nuclear localization and Vpx-mediated degradation.

    Science.gov (United States)

    Guo, Haoran; Wei, Wei; Wei, Zhenhong; Liu, Xianjun; Evans, Sean L; Yang, Weiming; Wang, Hong; Guo, Ying; Zhao, Ke; Zhou, Jian-Ying; Yu, Xiao-Fang

    2013-01-01

    The sterile alpha motif (SAM) and HD domain-containing protein-1 (SAMHD1) inhibits the infection of resting CD4+ T cells and myeloid cells by human and related simian immunodeficiency viruses (HIV and SIV). Vpx inactivates SAMHD1 by promoting its proteasome-dependent degradation through an interaction with CRL4 (DCAF1) E3 ubiquitin ligase and the C-terminal region of SAMHD1. However, the determinants in SAMHD1 that are required for Vpx-mediated degradation have not been well characterized. SAMHD1 contains a classical nuclear localization signal (NLS), and NLS point mutants are cytoplasmic and resistant to Vpx-mediated degradation. Here, we demonstrate that NLS-mutant SAMHD1 K11A can be rescued by wild-type SAMHD1, restoring its nuclear localization; consequently, SAMHD1 K11A became sensitive to Vpx-mediated degradation in the presence of wild-type SAMHD1. Surprisingly, deletion of N-terminal regions of SAMHD1, including the classical NLS, generated mutant SAMHD1 proteins that were again sensitive to Vpx-mediated degradation. Unlike SAMHD1 K11A, these deletion mutants could be detected in the nucleus. Interestingly, NLS-defective SAMHD1 could still bind to karyopherin-β1 and other nuclear proteins. We also determined that the linker region between the SAM and HD domain and the HD domain itself is important for Vpx-mediated degradation but not Vpx interaction. Thus, SAMHD1 contains an additional nuclear targeting mechanism in addition to the classical NLS. Our data indicate that multiple regions in SAMHD1 are critical for Vpx-mediated nuclear degradation and that association with Vpx is not sufficient for Vpx-mediated degradation of SAMHD1. Since the linker region and HD domain may be involved in SAMHD1 multimerization, our results suggest that SAMHD1 multimerization may be required for Vpx-mediation degradation.

  5. A Critical Examination of the Use of Trained Health Coaches to Decrease the Metabolic Syndrome for Participants of a Community-Based Diabetes Prevention and Management Program.

    Science.gov (United States)

    Lucke-Wold, Brandon; Shawley, Samantha; Ingels, John Spencer; Stewart, Jonathan; Misra, Ranjita

    2016-01-01

    The epidemic of obesity and diabetes in the United States poses major challenge to the prevention and management of chronic diseases. Furthermore, when this is viewed in other components of the metabolic syndrome (i.e., the burden of high cholesterol and hypertension), the prevalence of the metabolic syndrome continues to rise in the USA continued challenge is how to deal with this epidemic from a medical and public health standpoint. Community Based Participatory Research (CBPR) is a unique approach and offers a novel perspective for answering this challenge. A critical set of goals for CBPR is to address health disparities and social inequalities while getting community members engaged in all aspects of the research process. Utilizing the West Virginia Diabetes Prevention and Management Program and trained Health Coaches as a model, we discuss topics of consideration related to CBPR, involving trained health coaches, optimizing early adoption of healthy lifestyle behaviors, and enhancing participation. Through careful project planning and design, questions regarding disparities increasing susceptibility and preventive efforts within the community can be addressed successfully. These topics are part of a broader integration of theories such as participatory research, community engagement, and outcomes measurement. The understanding of the pathophysiology and epidemiology of the metabolic syndrome can help frame an appropriate strategy for establishing long-term community-wide changes that promote health. In order to continue to improve investigations for preventing the metabolic syndrome, it will be necessary to have aggressive efforts at the individual and population level for developing culturally sensitive programs that start early and are sustainable in practical environments such as the workplace. In this comprehensive review, we will discuss practical considerations related to project design, implementation, and how to measure effectiveness in regards to

  6. Isolation of a Genomic Region Affecting Most Components of Metabolic Syndrome in a Chromosome-16 Congenic Rat Model.

    Directory of Open Access Journals (Sweden)

    Lucie Šedová

    Full Text Available Metabolic syndrome is a highly prevalent human disease with substantial genomic and environmental components. Previous studies indicate the presence of significant genetic determinants of several features of metabolic syndrome on rat chromosome 16 (RNO16 and the syntenic regions of human genome. We derived the SHR.BN16 congenic strain by introgression of a limited RNO16 region from the Brown Norway congenic strain (BN-Lx into the genomic background of the spontaneously hypertensive rat (SHR strain. We compared the morphometric, metabolic, and hemodynamic profiles of adult male SHR and SHR.BN16 rats. We also compared in silico the DNA sequences for the differential segment in the BN-Lx and SHR parental strains. SHR.BN16 congenic rats had significantly lower weight, decreased concentrations of total triglycerides and cholesterol, and improved glucose tolerance compared with SHR rats. The concentrations of insulin, free fatty acids, and adiponectin were comparable between the two strains. SHR.BN16 rats had significantly lower systolic (18-28 mmHg difference and diastolic (10-15 mmHg difference blood pressure throughout the experiment (repeated-measures ANOVA, P < 0.001. The differential segment spans approximately 22 Mb of the telomeric part of the short arm of RNO16. The in silico analyses revealed over 1200 DNA variants between the BN-Lx and SHR genomes in the SHR.BN16 differential segment, 44 of which lead to missense mutations, and only eight of which (in Asb14, Il17rd, Itih1, Syt15, Ercc6, RGD1564958, Tmem161a, and Gatad2a genes are predicted to be damaging to the protein product. Furthermore, a number of genes within the RNO16 differential segment associated with metabolic syndrome components in human studies showed polymorphisms between SHR and BN-Lx (including Lpl, Nrg3, Pbx4, Cilp2, and Stab1. Our novel congenic rat model demonstrates that a limited genomic region on RNO16 in the SHR significantly affects many of the features of metabolic

  7. Low numbers of repeat units in variable number of tandem repeats (VNTR) regions of white spot syndrome virus are correlated with disease outbreaks

    NARCIS (Netherlands)

    Tran Thi Tuyet, H.; Zwart, M.P.; Phuong, N.T.; Jong, de M.C.M.; Vlak, J.M.

    2012-01-01

    White spot syndrome virus (WSSV) is the most important pathogen in shrimp farming systems worldwide including the Mekong Delta, Vietnam. The genome of WSSV is characterized by the presence of two major 'indel regions' found at ORF14/15 and ORF23/24 (WSSV-Thailand) and three regions with variable

  8. Implementing targeted region capture sequencing for the clinical detection of Alagille syndrome: An efficient and cost‑effective method.

    Science.gov (United States)

    Huang, Tianhong; Yang, Guilin; Dang, Xiao; Ao, Feijian; Li, Jiankang; He, Yizhou; Tang, Qiyuan; He, Qing

    2017-11-01

    Alagille syndrome (AGS) is a highly variable, autosomal dominant disease that affects multiple structures including the liver, heart, eyes, bones and face. Targeted region capture sequencing focuses on a panel of known pathogenic genes and provides a rapid, cost‑effective and accurate method for molecular diagnosis. In a Chinese family, this method was used on the proband and Sanger sequencing was applied to validate the candidate mutation. A de novo heterozygous mutation (c.3254_3255insT p.Leu1085PhefsX24) of the jagged 1 gene was identified as the potential disease‑causing gene mutation. In conclusion, the present study suggested that target region capture sequencing is an efficient, reliable and accurate approach for the clinical diagnosis of AGS. Furthermore, these results expand on the understanding of the pathogenesis of AGS.

  9. Restricted versus continued standard caloric intake during the management of refeeding syndrome in critically ill adults: a randomised, parallel-group, multicentre, single-blind controlled trial.

    Science.gov (United States)

    Doig, Gordon S; Simpson, Fiona; Heighes, Philippa T; Bellomo, Rinaldo; Chesher, Douglas; Caterson, Ian D; Reade, Michael C; Harrigan, Peter W J

    2015-12-01

    Equipoise exists regarding the benefits of restricting caloric intake during electrolyte replacement for refeeding syndrome, with half of intensive care specialists choosing to continue normal caloric intake. We aimed to assess whether energy restriction affects the duration of critical illness, and other measures of morbidity, compared with standard care. We did a randomised, multicentre, single-blind clinical trial in 13 hospital intensive care units (ICUs) in Australia (11 sites) and New Zealand (two sites). Adult critically ill patients who developed refeeding syndrome within 72 h of commencing nutritional support in the ICU were enrolled and allocated to receive continued standard nutritional support or protocolised caloric restriction. 1:1 computer-based randomisation was done in blocks of variable size, stratified by enrolment serum phosphate concentration (>0·32 mmol/L vs ≤0·32 mmol/L) and body-mass index (BMI; >18 kg/m(2)vs ≤18 kg/m(2)). The primary outcome was the number of days alive after ICU discharge, with 60 day follow-up, in a modified intention-to-treat population of all randomly allocated patients except those mistakenly enrolled. Days alive after ICU discharge was a composite outcome based on ICU length of stay, overall survival time, and mortality. The Refeeding Syndrome Trial was registered with the Australian and New Zealand Clinical Trials Registry (ANZCTR number 12609001043224). Between Dec 3, 2010, and Aug 13, 2014, we enrolled 339 adult critically ill patients: 170 were randomly allocated to continued standard nutritional support and 169 to protocolised caloric restriction. During the 60 day follow-up, the mean number of days alive after ICU discharge in 165 assessable patients in the standard care group was 39·9 (95% CI 36·4-43·7) compared with 44·8 (95% CI 40·9-49·1) in 166 assessable patients in the caloric restriction group (difference 4·9 days, 95% CI -2·3 to 13·6, p=0·19). Nevertheless, protocolised caloric

  10. Treatment of complex regional pain syndrome type 1 in a pediatric patient using the lidocaine patch 5%: a case report

    Directory of Open Access Journals (Sweden)

    Steven G Frost, MD

    2003-09-01

    Full Text Available Background: Successful treatment of complex regional pain syndrome type 1 (CRPS-1 requires a coordinated, multidisciplinary approach. Physical rehabilitation is an important component of long-term treatment. Unfortunately, patients with significant allodynia or hyperalgesia characteristic of CRPS-1 often have difficulty progressing through a physical therapy (PT regimen. In most adults with CRPS-1, the treatment of choice is PO opioids. Objective: This article presents a case report of the use of the lidocaine patch 5%, a targeted peripheral analgesic, in a pediatric patient and its effects on reducing pain, improving the patient's overall attitude, and facilitating compliance with ongoing PT. Results: A 10-year-old girl developed CRPS-1 after arthroscopic surgery for a sprained ankle. Attempts at PT were unsuccessful due to inadequate pain relief from various treatment modalities. Therapy with the lidocaine patch 5% was initiated and resulted in significant pain relief, improvements in the patient's attitude, and progress with PT. Conclusion: This case report of a child with CRPS-1 showed that therapy with lidocaine patch 5% may be efficacious in treating children with pain resulting from CRPS-1, thereby increasing the success of PT. Keywords: complex regional pain syndrome, lidocaine patch 5%, targeted peripheral analgesic, pediatrics

  11. Electroencephalographic evoked pain response is suppressed by spinal cord stimulation in complex regional pain syndrome: a case report.

    Science.gov (United States)

    Hylands-White, Nicholas; Duarte, Rui V; Beeson, Paul; Mayhew, Stephen D; Raphael, Jon H

    2016-12-01

    Pain is a subjective response that limits assessment. The purpose of this case report was to explore how the objectivity of the electroencephalographic response to thermal stimuli would be affected by concurrent spinal cord stimulation. A patient had been implanted with a spinal cord stimulator for the management of complex regional pain syndrome of both hands for 8 years. Following ethical approval and written informed consent we induced thermal stimuli using the Medoc PATHWAY Pain & Sensory Evaluation System on the right hand of the patient with the spinal cord stimulator switched off and with the spinal cord stimulator switched on. The patient reported a clinically significant reduction in thermal induced pain using the numerical rating scale (71.4 % reduction) with spinal cord stimulator switched on. Analysis of electroencephalogram recordings indicated the occurrence of contact heat evoked potentials (N2-P2) with spinal cord stimulator off, but not with spinal cord stimulator on. This case report suggests that thermal pain can be reduced in complex regional pain syndrome patients with the use of spinal cord stimulation and offers objective validation of the reported outcomes with this treatment.

  12. Host persistence or extinction from emerging infectious disease: insights from white-nose syndrome in endemic and invading regions.

    Science.gov (United States)

    Hoyt, Joseph R; Langwig, Kate E; Sun, Keping; Lu, Guanjun; Parise, Katy L; Jiang, Tinglei; Frick, Winifred F; Foster, Jeffrey T; Feng, Jiang; Kilpatrick, A Marm

    2016-03-16

    Predicting species' fates following the introduction of a novel pathogen is a significant and growing problem in conservation. Comparing disease dynamics between introduced and endemic regions can offer insight into which naive hosts will persist or go extinct, with disease acting as a filter on host communities. We examined four hypothesized mechanisms for host-pathogen persistence by comparing host infection patterns and environmental reservoirs for Pseudogymnoascus destructans (the causative agent of white-nose syndrome) in Asia, an endemic region, and North America, where the pathogen has recently invaded. Although colony sizes of bats and hibernacula temperatures were very similar, both infection prevalence and fungal loads were much lower on bats and in the environment in Asia than North America. These results indicate that transmission intensity and pathogen growth are lower in Asia, likely due to higher host resistance to pathogen growth in this endemic region, and not due to host tolerance, lower transmission due to smaller populations, or lower environmentally driven pathogen growth rate. Disease filtering also appears to be favouring initially resistant species in North America. More broadly, determining the mechanisms allowing species persistence in endemic regions can help identify species at greater risk of extinction in introduced regions, and determine the consequences for disease dynamics and host-pathogen coevolution. © 2016 The Author(s).

  13. Equivalent dose, effective dose and risk assessment from panoramic radiography to the critical organs of head and neck region

    International Nuclear Information System (INIS)

    Cho, Bong Hae; Nah, Kyung Soo; Lee, Ae Ryeon

    1995-01-01

    The purpose of this study was to evaluate the equivalent and effective dose, and estimate radiation risk to the critical organs of head and neck region from the use of adult and child mode in panoramic radiography. The results were as follows. 1. The salivary glands showed the highest equivalent and effective dose in adult and child mode. The equivalent and effective dose in adult mode were 837 μSv and 20.93 μSv, those in child mode were 462 μSv and 11.54 μSv, respectively. 2. Total effective doses to the critical head and neck organs were estimated 34.2l μSv in adult mode, 20.14 μSv in child mode. From these data, the probabilities of stochastic effect from adult and child mode were 2.50xl0 -6 and 1.47x10 -6 3. The other remainder showed the greatest risk of fatal cancer. The risk estimate were 4.5 and 2.7 fatal malignancies in adult and child mode from million examinations. The bone marrow and thyroid gland showed about 0.1 fatal cancer in adult. and child mode from these examinations.

  14. Complexity of the 5′ Untranslated Region of EIF4A3, a Critical Factor for Craniofacial and Neural Development

    Directory of Open Access Journals (Sweden)

    Gabriella S. P. Hsia

    2018-04-01

    Full Text Available Repeats in coding and non-coding regions have increasingly been associated with many human genetic disorders, such as Richieri-Costa-Pereira syndrome (RCPS. RCPS, mostly characterized by midline cleft mandible, Robin sequence and limb defects, is an autosomal-recessive acrofacial dysostosis mainly reported in Brazilian patients. This disorder is caused by decreased levels of EIF4A3, mostly due to an increased number of repeats at the EIF4A3 5′UTR. EIF4A3 5′UTR alleles are CG-rich and vary in size and organization of three types of motifs. An exclusive allelic pattern was identified among affected individuals, in which the CGCA-motif is the most prevalent, herein referred as “disease-associated CGCA-20nt motif.” The origin of the pathogenic alleles containing the disease-associated motif, as well as the functional effects of the 5′UTR motifs on EIF4A3 expression, to date, are entirely unknown. Here, we characterized 43 different EIF4A3 5′UTR alleles in a cohort of 380 unaffected individuals. We identified eight heterozygous unaffected individuals harboring the disease-associated CGCA-20nt motif and our haplotype analyses indicate that there are more than one haplotype associated with RCPS. The combined analysis of number, motif organization and haplotypic diversity, as well as the observation of two apparently distinct haplotypes associated with the disease-associated CGCA-20nt motif, suggest that the RCPS alleles might have arisen from independent unequal crossing-over events between ancient alleles at least twice. Moreover, we have shown that the number and sequence of motifs in the 5′UTR region is associated with EIF4A3 repression, which is not mediated by CpG methylation. In conclusion, this study has shown that the large number of repeats in EIF4A3 does not represent a dynamic mutation and RCPS can arise in any population harboring alleles with the CGCA-20nt motif. We also provided further evidence that EIF4A3 5′UTR is a

  15. Complexity of the 5' Untranslated Region of EIF4A3, a Critical Factor for Craniofacial and Neural Development.

    Science.gov (United States)

    Hsia, Gabriella S P; Musso, Camila M; Alvizi, Lucas; Brito, Luciano A; Kobayashi, Gerson S; Pavanello, Rita C M; Zatz, Mayana; Gardham, Alice; Wakeling, Emma; Zechi-Ceide, Roseli M; Bertola, Debora; Passos-Bueno, Maria Rita

    2018-01-01

    Repeats in coding and non-coding regions have increasingly been associated with many human genetic disorders, such as Richieri-Costa-Pereira syndrome (RCPS). RCPS, mostly characterized by midline cleft mandible, Robin sequence and limb defects, is an autosomal-recessive acrofacial dysostosis mainly reported in Brazilian patients. This disorder is caused by decreased levels of EIF4A3 , mostly due to an increased number of repeats at the EIF4A3 5'UTR. EIF4A3 5'UTR alleles are CG-rich and vary in size and organization of three types of motifs. An exclusive allelic pattern was identified among affected individuals, in which the CGCA-motif is the most prevalent, herein referred as "disease-associated CGCA-20nt motif." The origin of the pathogenic alleles containing the disease-associated motif, as well as the functional effects of the 5'UTR motifs on EIF4A3 expression, to date, are entirely unknown. Here, we characterized 43 different EIF4A3 5'UTR alleles in a cohort of 380 unaffected individuals. We identified eight heterozygous unaffected individuals harboring the disease-associated CGCA-20nt motif and our haplotype analyses indicate that there are more than one haplotype associated with RCPS. The combined analysis of number, motif organization and haplotypic diversity, as well as the observation of two apparently distinct haplotypes associated with the disease-associated CGCA-20nt motif, suggest that the RCPS alleles might have arisen from independent unequal crossing-over events between ancient alleles at least twice. Moreover, we have shown that the number and sequence of motifs in the 5'UTR region is associated with EIF4A3 repression, which is not mediated by CpG methylation. In conclusion, this study has shown that the large number of repeats in EIF4A3 does not represent a dynamic mutation and RCPS can arise in any population harboring alleles with the CGCA-20nt motif. We also provided further evidence that EIF4A3 5'UTR is a regulatory region and the size and

  16. Comparative mapping of DNA markers from the familial Alzheimer disease and Down syndrome regions of human chromosome 21 to mouse chromosomes 16 and 17

    Energy Technology Data Exchange (ETDEWEB)

    Cheng, S.V.; Nadeau, J.H.; Tanzi, R.E.; Watkins, P.C.; Jagadesh, J.; Taylor, B.A.; Haines, J.L.; Sacchi, N.; Gusella, J.F. (Harvard Medical School, Boston, MA (USA))

    1988-08-01

    Mouse trisomy 16 has been proposed as an animal model of Down syndrome (DS), since this chromosome contains homologues of several loci from the q22 band of human chromosome 21. The recent mapping of the defect causing familial Alzheimer disease (FAD) and the locus encoding the Alzheimer amyloid {beta} precursor protein (APP) to human chromosome 21 has prompted a more detailed examination of the extent of conservation of this linkage group between the two species. Using anonymous DNA probes and cloned genes from human chromosome 21 in a combination of recombinant inbred and interspecific mouse backcross analyses, the authors have established that the linkage group shared by mouse chromosome 16 includes not only the critical DS region of human chromosome 21 but also the APP gene and FAD-linked markers. Extending from the anonymous DNA locus D21S52 to ETS2, the linkage map of six loci spans 39% recombination in man but only 6.4% recombination in the mouse. A break in synteny occurs distal to ETS2, with the homologue of the human marker D21S56 mapping to mouse chromosome 17. Conservation of the linkage relationships of markers in the FAD region suggests that the murine homologue of the FAD locus probably maps to chromosome 16 and that detailed comparison of the corresponding region in both species could facilitate identification of the primary defect in this disorder. The break in synteny between the terminal portion of human chromosome 21 and mouse chromosome 16 indicates, however, that mouse trisomy 16 may not represent a complete model of DS.

  17. Comparative mapping of DNA markers from the familial Alzheimer disease and Down syndrome regions of human chromosome 21 to mouse chromosomes 16 and 17

    International Nuclear Information System (INIS)

    Cheng, S.V.; Nadeau, J.H.; Tanzi, R.E.; Watkins, P.C.; Jagadesh, J.; Taylor, B.A.; Haines, J.L.; Sacchi, N.; Gusella, J.F.

    1988-01-01

    Mouse trisomy 16 has been proposed as an animal model of Down syndrome (DS), since this chromosome contains homologues of several loci from the q22 band of human chromosome 21. The recent mapping of the defect causing familial Alzheimer disease (FAD) and the locus encoding the Alzheimer amyloid β precursor protein (APP) to human chromosome 21 has prompted a more detailed examination of the extent of conservation of this linkage group between the two species. Using anonymous DNA probes and cloned genes from human chromosome 21 in a combination of recombinant inbred and interspecific mouse backcross analyses, the authors have established that the linkage group shared by mouse chromosome 16 includes not only the critical DS region of human chromosome 21 but also the APP gene and FAD-linked markers. Extending from the anonymous DNA locus D21S52 to ETS2, the linkage map of six loci spans 39% recombination in man but only 6.4% recombination in the mouse. A break in synteny occurs distal to ETS2, with the homologue of the human marker D21S56 mapping to mouse chromosome 17. Conservation of the linkage relationships of markers in the FAD region suggests that the murine homologue of the FAD locus probably maps to chromosome 16 and that detailed comparison of the corresponding region in both species could facilitate identification of the primary defect in this disorder. The break in synteny between the terminal portion of human chromosome 21 and mouse chromosome 16 indicates, however, that mouse trisomy 16 may not represent a complete model of DS

  18. "How Can a Chord Be Weird if It Expresses Your Soul?" Some Critical Reflections on the Diagnosis of Aspergers Syndrome

    Science.gov (United States)

    Moloney, Paul

    2010-01-01

    This paper questions the way in which the diagnosis of Aspergers syndrome has come to be widely accepted and used as an essentially medical category. It does so by drawing upon sociological and historical analyses of society, psychiatry and psychology, as well as the writings of service users, other practitioners in the autistic spectrum disorder…

  19. Equivalence of chain conformations in the surface region of a polymer melt and a single Gaussian chain under critical conditions.

    Science.gov (United States)

    Skvortsov, A M; Leermakers, F A M; Fleer, G J

    2013-08-07

    In the melt polymer conformations are nearly ideal according to Flory's ideality hypothesis. Silberberg generalized this statement for chains in the interfacial region. We check the Silberberg argument by analyzing the conformations of a probe chain end-grafted at a solid surface in a sea of floating free chains of concentration φ by the self-consistent field (SCF) method. Apart from the grafting, probe chain and floating chains are identical. Most of the results were obtained for a standard SCF model with freely jointed chains on a six-choice lattice, where immediate step reversals are allowed. A few data were generated for a five-choice lattice, where such step reversals are forbidden. These coarse-grained models describe the equilibrium properties of flexible atactic polymer chains at the scale of the segment length. The concentration was varied over the whole range from φ = 0 (single grafted chain) to φ = 1 (probe chain in the melt). The number of contacts with the surface, average height of the free end and its dispersion, average loop and train length, tail size distribution, end-point and overall segment distributions were calculated for a grafted probe chain as a function of φ, for several chain lengths and substrate∕polymer interactions, which were varied from strong repulsion to strong adsorption. The computations show that the conformations of the probe chain in the melt do not depend on substrate∕polymer interactions and are very similar to the conformations of a single end-grafted chain under critical conditions, and can thus be described analytically. When the substrate∕polymer interaction is fixed at the value corresponding to critical conditions, all equilibrium properties of a probe chain are independent of φ, over the whole range from a dilute solution to the melt. We believe that the conformations of all flexible chains in the surface region of the melt are close to those of an appropriate single chain in critical conditions, provided

  20. Comparisons of metabolic syndrome definitions in four populations of the Asia-Pacific region

    DEFF Research Database (Denmark)

    Lee, C.M.; Huxley, R.R.; Woodward, M.

    2008-01-01

    BACKGROUND: To compare the prevalence of metabolic syndrome (MetS) by four MetS definitions in four Asia-Pacific populations, and to compare the prevalence of individual metabolic components. METHODS: Population-based cross-sectional studies from Australia, Japan, Korea, and Samoa were used...... for EGIR. There were marked differences in the prevalence of MetS between the sexes, with no systematic pattern, and between the prevalences of individual metabolic components. CONCLUSIONS: Differences in the prevalence of MetS and its components, using the various definitions, both within and between...

  1. Regional ventilation/perfusion mismatch pattern in patient with Swyer James (MacLeod′s syndrome

    Directory of Open Access Journals (Sweden)

    Sait Sager

    2014-01-01

    Full Text Available Swyer James (McLeod′s syndrome (SJMS is an uncommon disease, which occurs as a result of childhood bronchiolitis obliterans. Patients may not be diagnosed until later in their life. A 46-year-old man underwent ventilation/perfusion scintigraphy for acute onset of dyspnea. The scan showed markedly diminished ventilation and perfusion unilaterally on the right middle and inferior lobes. However, mismatched ventilation-perfusion pattern was shown on the upper right lobe, which was consistent with pulmonary embolism. Unilaterally matched ventilation/perfusion defect can see in SJMS in lung scintigraphy; however, when pulmoner embolism may accompany, scintigraphy should be carefully examined.

  2. CAGE-defined promoter regions of the genes implicated in Rett Syndrome

    DEFF Research Database (Denmark)

    Vitezic, Morana; Bertin, Nicolas; Andersson, Robin

    2014-01-01

    BACKGROUND: Mutations in three functionally diverse genes cause Rett Syndrome. Although the functions of Forkhead box G1 (FOXG1), Methyl CpG binding protein 2 (MECP2) and Cyclin-dependent kinase-like 5 (CDKL5) have been studied individually, not much is known about their relation to each other...... reveal the predominantly used transcription start sites (TSSs) for each gene including novel transcription start sites for FOXG1. We show that FOXG1 expression is poorly correlated with the expression of MECP2 and CDKL5. We identify promoter shapes for each TSS, the predicted location of enhancers...

  3. Delayed diagnosis and worsening of pain following orthopedic surgery in patients with complex regional pain syndrome (CRPS).

    Science.gov (United States)

    Lunden, Lars K; Kleggetveit, Inge P; Jørum, Ellen

    2016-04-01

    Complex regional pain syndrome (CRPS) is a serious and disabling chronic pain condition, usually occurring in a limb. There are two main types, CRPS 1 with no definite nerve lesion and CRPS 2 with an identified nerve lesion. CRPS 1 and 2 may occur following an injury (frequently following fractures), surgery or without known cause. An early diagnosis and start of adequate treatment is considered desirable for patients with CRPS. From the clinical experience of the principal investigator, it became apparent that CRPS often remained undiagnosed and that the clinical conditions of many patients seemed to be worsened following orthopedic surgery subsequent to the initial eliciting event. The aim of the present retrospective study of 55 patients, all diagnosed with either CRPS 1 or 2, was to evaluate the time from injury until diagnosis of CRPS and the effect on pain of orthopedic surgical intervention subsequent to the original injury/surgery. Clinical symptoms with an emphasis on pain were assessed by going through the patients' records and by information given during the investigation at Oslo University Hospital, where the patients also were examined clinically and with EMG/neurography. Alteration in pain was evaluated in 27 patients who underwent orthopedic surgery subsequent to the eliciting injury. Of a total of 55 patients, 28 women and 27 men (mean age 38.7 (SD 12.3), 38 patients were diagnosed with CRPS type 1, and 17 with CRPS type 2. Mean time before diagnosis was confirmed was 3.9 years (SD1.42, range 6 months-10 years). The eliciting injuries for both CRPS type 1 and type 2 were fractures, squeeze injuries, blunt injuries, stretch accidents and surgery. A total of 27 patients (14 men and 13 women) were operated from one to 12 times at a later stage (from 6 months to several years) following the initial injury or any primary operation because of fracture. A total of 22 patients reported a worsening of pain following secondary surgical events, while four

  4. Tumour MLH1 promoter region methylation testing is an effective pre-screen for Lynch Syndrome (HNPCC)

    Science.gov (United States)

    Newton, K; Jorgensen, NM; Wallace, AJ; Buchanan, DD; Lalloo, F; McMahon, RFT; Hill, J; Evans, DG

    2016-01-01

    Background & Aims Lynch syndrome patients have DNA mismatch repair deficiency and up to 80% life-time risk of colorectal cancer. Screening of mutation carriers reduces colorectal cancer incidence and mortality. Selection for constitutional mutation testing relies on family history (Amsterdam and Bethesda Guidelines) and tumour derived biomarkers. Initial biomarker analysis uses mismatch repair protein immunohistochemistry and microsatellite instability. Abnormalities in either identify mismatch repair deficiency but do not differentiate sporadic epigenetic defects, due to MLH1 promoter region methylation (13% of CRCs) from Lynch Syndrome (4% of CRCs). A diagnostic biomarker capable of making this distinction would be valuable. This study compared two biomarkers in tumours with mismatch repair deficiency; quantification of methylation of the MLH1 promoter region using a novel assay and BRAF c.1799T>A, p.(Val600Glu) mutation status in the identification of constitutional mutations. Methods Tumour DNA was extracted (FFPE tissue) and pyrosequencing used to test for MLH1 promoter methylation and presence of the BRAF c.1799T>A, p.(Val600Glu) mutation 71 CRCs from individuals with pathogenic MLH1 mutations and 73 CRCs with sporadic MLH1 loss. Specificity and sensitivity was compared. Findings Unmethylated MLH1 promoter: sensitivity 94.4% (95% CI 86.2–98.4%), specificity 87.7% (95% CI 77.9–94.2%), Wild-type BRAF (codon 600): sensitivity 65.8% (95% CI 53.7–76.5%), specificity 98.6% (95% CI 92.4–100.0%) for the identification of those with pathogenic MLH1 mutations. Conclusions Quantitative MLH1 promoter region methylation using pyrosequencing is superior to BRAF codon 600 mutation status in identifying constitutional mutations in mismatch repair deficient tumours. PMID:25280751

  5. Intra-abdominal hypertension and abdominal compartment syndrome in association with ruptured abdominal aortic aneurysm in the endovascular era: vigilance remains critical.

    Science.gov (United States)

    Bozeman, Matthew C; Ross, Charles B

    2012-01-01

    Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are common complications of ruptured abdominal aortoiliac aneurysms (rAAAs) and other abdominal vascular catastrophes even in the age of endovascular therapy. Morbidity and mortality due to systemic inflammatory response syndrome (SIRS) and multiple organ failure (MOF) are significant. Recognition and management of IAH are key critical care measures which may decrease morbidity and improve survival in these vascular patients. Two strategies have been utilized: expectant management with prompt decompressive laparotomy upon diagnosis of threshold levels of IAH versus prophylactic, delayed abdominal closure based upon clinical parameters at the time of initial repair. Competent management of the abdominal wound with preservation of abdominal domain is also an important component of the care of these patients. In this review, we describe published experience with IAH and ACS complicating abdominal vascular catastrophes, experience with ACS complicating endovascular repair of rAAAs, and techniques for management of the abdominal wound. Vigilance and appropriate management of IAH and ACS remains critically important in decreasing morbidity and optimizing survival following catastrophic intra-abdominal vascular events.

  6. Intravenous regional block is similar to sympathetic ganglion block for pain management in patients with complex regional pain syndrome type I

    Directory of Open Access Journals (Sweden)

    M.S.A. Nascimento

    2010-12-01

    Full Text Available Sympathetic ganglion block (SGB or intravenous regional block (IVRB has been recommended for pain management in patients with complex regional pain syndrome type I (CRPS-I. Forty-five patients were initially selected but only 43 were accepted for the study. The present study evaluated the efficacy of IVRB produced by combining 70 mg lidocaine with 30 µg clonidine (14 patients, 1 male/13 females, age range: 27-50 years versus SGB produced by the injection of 70 mg lidocaine alone (14 patients, 1 male/13 females, age range: 27-54 years or combined with 30 µg clonidine (15 patients, 1 male/14 females, age range: 25-50 years into the stellate ganglion for pain management in patients with upper extremity CRPS-I. Each procedure was repeated five times at 7-day intervals, and pain intensity and duration were measured using a visual analog scale immediately before each procedure. A progressive and significant reduction in pain scores and a significant increase in the duration of analgesia were observed in all groups following the first three blocks, but no further improvement was obtained following the last two blocks. Drowsiness, the most frequent side effect, and dry mouth occurred only in patients submitted to SGB with lidocaine combined with clonidine. The three methods were similar regarding changes in pain intensity and duration of analgesia. However, IVRB seems to be preferable to SGB due to its easier execution and lower risk of undesirable effects.

  7. Nance-Horan syndrome: localization within the region Xp21.1-Xp22.3 by linkage analysis.

    Science.gov (United States)

    Stambolian, D; Lewis, R A; Buetow, K; Bond, A; Nussbaum, R

    1990-07-01

    Nance-Horan Syndrome (NHS) or X-linked cataract-dental syndrome (MIM 302350) is a disease of unknown pathogenesis characterized by congenital cataracts and dental anomalies. We performed linkage analysis in three kindreds with NHS by using six RFLP markers between Xp11.3 and Xp22.3. Close linkage was found between NHS and polymorphic loci DXS43 (theta = 0 with lod score 2.89), DXS41 (theta = 0 with lod score 3.44), and DXS67 (theta = 0 with lod score 2.74), defined by probes pD2, p99-6, and pB24, respectively. Recombinations were found with the marker loci DXS84 (theta = .04 with lod score 4.13), DXS143 (theta = .06 with lod score 3.11) and DXS7 (theta = .09 with lod score 1.68). Multipoint linkage analysis determined the NHS locus to be linked completely to DXS41 (lod score = 7.07). Our linkage results, combined with analysis of Xp interstitial deletions, suggest that the NHS locus is located within or close to the Xp22.1-Xp22.2 region.

  8. Clinical insights on Tolosa Hunt syndrome: A multidisciplinary approach on neurological-related symptomatology in maxillofacial region

    Directory of Open Access Journals (Sweden)

    Zafar Ali Khan

    2018-02-01

    Full Text Available Background Tolosa–Hunt syndrome (THS related neurological symptoms are described in literature as “unilateral”, “recurrent”, “episodic”, “intense”, “severe”, “lancinating” or “stabbing” pain on the upper face and forehead and may be misdiagnosed due to the similarity of few symptoms and a significant number of common characteristics between both conditions. Aims The aim of this brief report is to indicate some important clinical insights related to Tolosa Hunt syndrome, and to give a frank account on the multidisciplinary approach on neurological-related symptomatology in maxillofacial region. Methods We analysed a selection of patients with such clinical picture. To better describe the proper management of clinical cases, we report a 50-year-old female reporting an history of two years of recurrent, severe stabbing pain around the right eye, prominence of her cheek and forehead. Her general dentist first mistakenly diagnosed toothache and, thus, it was subsequently misdiagnosed the trigeminal neuropathy (TN. Results Reported exemplificative case presented a mild ptosis, diplopia of the right eye, corneal reflex loss, paresthesia and hyperesthesia of upper part of left side of face. Magnetic resonance imaging (MRI findings were suggestive of THS. Complete resolution of symptoms was achieved with oral Prednisolone and constant monitoring of symptoms. Conclusion THS may be added to the long list of differential diagnosis of TN and general dentist and oral surgeons should be informed about such rare causes of facial pain through continued medical education programs.

  9. Prevalence and trends of metabolic syndrome among adults in the asia-pacific region: a systematic review

    Directory of Open Access Journals (Sweden)

    P. Ranasinghe

    2017-01-01

    Full Text Available Abstract Background The Asia-Pacific region is home to nearly half of the world’s population. The region has seen a recent rapid increase in the prevalence of obesity, type-2 diabetes and cardiovascular disease. The present systematic review summarizes the recent prevalence and trends of Metabolic Syndrome (MetS among adults in countries of the Asia-Pacific Region. Methods Data on MetS in Asia-Pacific countries were obtained using a stepwise process by searching the online Medline database using MeSH terms ‘Metabolic Syndrome X’ and ‘Epidemiology/EP’. For the purpose of describing prevalence data for the individual countries, studies that were most recent, nationally representative or with the largest sample size were included. When evaluating secular trends in prevalence in a country we only considered studies that evaluated the temporal change in prevalence between similar populations, prospective studies based on the same population or National surveys conducted during different time periods. Results This literature search yielded a total of 757 articles, and five additional article were identified by screening of reference lists. From this total, 18 studies were eligible to be included in the final analysis. Of the 51 Asia-Pacific countries (WHO we only located data for 15. There was wide between country variation in prevalence of MetS. A national survey from Philippines conducted in 2003 revealed the lowest reported prevalence of 11.9% according to NCEP ATP III criteria. In contrast, the highest recorded prevalence in the region (49.0% came from a study conducted in urban Pakistan (Karachchi, 2004. Most studies reported a higher prevalence of MetS in females and urban residents. Data on secular trends were available for China, South Korea and Taiwan. An increase in the prevalence of MetS was observed in all three countries. Conclusion Despite differences in methodology, diagnostic criteria and age of subjects studied, the Asia

  10. Integrated GIS and multivariate statistical analysis for regional scale assessment of heavy metal soil contamination: A critical review

    International Nuclear Information System (INIS)

    Hou, Deyi; O'Connor, David; Nathanail, Paul; Tian, Li; Ma, Yan

    2017-01-01

    analysis (PCA) and cluster analysis (CA). - Highlights: • Critically reviewed field studies of regional distribution of heavy metal in soil. • Reviewed studies combined GIS with multivariate statistical analysis. • Summarized the most common sampling strategies, GIS method, and multivariate technique. • Identified determining and correlating factors for soil heavy metals. • Discussed major natural and anthropogenic sources of soil heavy metals. - The integration of GIS and multivariate statistical analysis is a valuable tool in studying the regional distribution of heavy metals in soil.

  11. The phase transition of the first order in the critical region of the gas-liquid system

    Directory of Open Access Journals (Sweden)

    I.R. Yukhnovskii

    2014-12-01

    Full Text Available This is a summarising investigation of the events of the phase transition of the first order that occur in the critical region below the liquid-gas critical point. The grand partition function has been completely integrated in the phase-space of the collective variables. The basic density measure is the quartic one. It has the form of the exponent function with the first, second, third and fourth degree of the collective variables. The problem has been reduced to the Ising model in an external field, the role of which is played by the generalised chemical potential μ*. The line μ*(η =0, where η is the density, is the line of the phase transition. We consider the isothermal compression of the gas till the point where the phase transition on the line μ*(η =0 is reached. When the path of the pressing reaches the line μ* =0 in the gas medium, a droplet of liquid springs up. The work for its formation is obtained, the surface-tension energy is calculated. On the line μ* =0 we have a two-phase system: the gas and the liquid (the droplet one. The equality of the gas and of the liquid chemical potentials is proved. The process of pressing is going on. But the pressure inside the system has stopped, two fixed densities have arisen: one for the gas-phase ηG=ηc(1-d/2 and the other for the liquid-phase ηL=ηc(1+d/2 (symmetrically to the rectlinear diameter, where ηc=0.13044 is the critical density. Starting from that moment the external pressure works as a latent work of pressure. Its value is obtained. As a result, the gas-phase disappears and the whole system turns into liquid. The jump of the density is equal to ηc d, where d=(D/2G1/2 ~ τν/2. D and G are coefficients of the Hamiltonian in the last cell connected with the renormalisation-group symmetry. The equation of state is written.

  12. Treatment of chronic regional pain syndrome type 1 with palmitoylethanolamide and topical ketamine cream: modulation of nonneuronal cells

    Directory of Open Access Journals (Sweden)

    Keppel Hesselink JM

    2013-03-01

    Full Text Available Jan M Keppel Hesselink,1 David J Kopsky21Institute for Neuropathic Pain, Bosch en Duin, The Netherlands; 2Institute for Neuropathic Pain, Amsterdam, The NetherlandsAbstract: Chronic regional pain syndrome (CRPS can be intractable to treat and patients sometimes suffer for many years. Therefore, new treatment strategies are needed to alleviate symptoms in CRPS patients. This case report describes a patient suffering from intractable CRPS type 1 for 13 years. Due to her swollen painful feet and left knee she is wheelchair-bound. The combination of palmitoylethanolamide and ketamine 10% cream reduced her pain by more than 50% after 1 month of treatment, and a marked reduction in swelling and skin discoloration was noticed. Furthermore, she could walk independently again and she experienced no side effects. Thus, palmitoylethanolamide and topical ketamine could be a combination therapy option for treating CRPS patients.Keywords: palmitoylethanolamide, ketamine, cream, CRPS, endocannabinoid, sudeck, mast cells

  13. Case Study of High-Dose Ketamine for Treatment of Complex Regional Pain Syndrome in the Pediatric Intensive Care Unit.

    Science.gov (United States)

    Pasek, Tracy Ann; Crowley, Kelli; Campese, Catherine; Lauer, Rachel; Yang, Charles

    2017-06-01

    Complex regional pain syndrome (CRPS) is a life-altering and debilitating chronic pain condition. The authors are presenting a case study of a female who received high-dose ketamine for the management of her CRPS. The innovative treatment lies not only within the pharmacologic management of her pain, but also in the fact that she was the first patient to be admitted to our pediatric intensive care unit solely for pain control. The primary component of the pharmacotherapy treatment strategy plan was escalating-dose ketamine infusion via patient-controlled-analgesia approved by the pharmacy and therapeutics committee guided therapy for this patient. The expertise of advanced practice nurses blended exquisitely to ensure patient and family-centered care and the coordination of care across the illness trajectory. The patient experienced positive outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. [Diagnostics of broncho-obstructive states in children with wheezing syndrome and who live in ecologically non-friendly regions].

    Science.gov (United States)

    Antypkin, Iu H; Velychko, M I; Arabs'ka, L P; Barteniev, S H; Pavlovs'kyĭ, V S; Dardyns'ka, I V

    2008-01-01

    Disorders found by Spirography have been analysed. These data were obtained from 137 children with bronchial asthma and with wheezing syndrome and who live in ecologically not unfavorable regions of Ukraine. Decrease in patency of airways of small and big caliber was detected in patients with bronchial asthma from Dneprodzerzhinsk and the middle and Decrease in patency of airways of middle and small caliber small caliber of patients from Mariupol. The pharmacological test with salbuthamol demonstrated considerable changes in the reactivity of bronchial apparatus that shows necessity of administration of drugs correction in children with bronchial asthma and necessity to detail indications for this correction to patients with an isolated decreased patency of airways of big caliber.

  15. Cervical Epidural Anaesthesia for Radical Mastectomy and Chronic Regional Pain Syndrome of Upper Limb - A Case Report

    Directory of Open Access Journals (Sweden)

    Ashok Jadon

    2009-01-01

    Full Text Available A 47-yrs-female patient presented with carcinoma right breast, swelling and allodynia of right upper limb. radical mastectomy with axillary clearance and skin grafting was done under cervical epidural anaesthesia through 18G epidural catheter placed at C6/C7 level. Postoperative analgesia and rehabilitation of affected right upper limb was managed by continuous epidural infusion of 0.125% bupivacaine and 2.5 µg/ml -1 clonidine solution through epidu-ral catheter for 5 days and physiotherapy. This case report highlights the usefulness of cervical epidural analgesia in managing a complex situation of carcinoma breast with associated periarthitis of shoulder joint and chronic regional pain syndrome (CRPS of right upper limb.

  16. Regulation of peripheral blood flow in Complex Regional Pain Syndrome: clinical implication for symptomatic relief and pain management

    Directory of Open Access Journals (Sweden)

    Coderre Terence J

    2009-09-01

    Full Text Available Abstract Background During the chronic stage of Complex Regional Pain Syndrome (CRPS, impaired microcirculation is related to increased vasoconstriction, tissue hypoxia, and metabolic tissue acidosis in the affected limb. Several mechanisms may be responsible for the ischemia and pain in chronic cold CPRS. Discussion The diminished blood flow may be caused by either sympathetic dysfunction, hypersensitivity to circulating catecholamines, or endothelial dysfunction. The pain may be of neuropathic, inflammatory, nociceptive, or functional nature, or of mixed origin. Summary The origin of the pain should be the basis of the symptomatic therapy. Since the difference in temperature between both hands fluctuates over time in cold CRPS, when in doubt, the clinician should prioritize the patient's report of a persistent cold extremity over clinical tests that show no difference. Future research should focus on developing easily applied methods for clinical use to differentiate between central and peripheral blood flow regulation disorders in individual patients.

  17. Intrathecal Bupivacaine Monotherapy with a Retrograde Catheter for the Management of Complex Regional Pain Syndrome of the Lower Extremity.

    Science.gov (United States)

    McRoberts, W Porter; Apostol, Catalina; Haleem, Abdul

    2016-01-01

    Complex regional pain syndrome (CRPS) presents a therapeutic challenge due to its many presentations and multifaceted pathophysiology. There is no approved treatment algorithm and clinical interventions are often applied empirically. In cases of CRPS where symptoms are localized to an extremity, a targeted treatment is indicated. We describe the use of intrathecal bupivacaine monotherapy, delivered through a retrograde catheter, in the treatment of CRPS affecting the lower extremity. The patient, a 57-year-old woman with a history of failed foot surgery, was seen in our office after 2 years of ineffective treatments with local blocks and neurolytic procedures. We advanced therapy to moderately invasive procedures with an emphasis on neuromodulation. A combined central and peripheral stimulation technique that initially provided 75% pain relief, failed to provide lasting analgesia. We proceeded with an intrathecal pump implant. Based on the results of dorsal root ganglion (DRG) mapping, L5-S1 was identified as the optimal target for therapy and a retrograde catheter was placed at this level. Various intrathecal medications were tested individually. An intrathecal morphine trial was ineffective (visual analog scale [VAS] 7), while intrathecal clonidine provided excellent pain relief (VAS 0) that was limited by severe side effects. Bupivacaine provided 100% analgesia with tolerable side effects (lower extremity weakness and minor bladder incontinence) and was selected for intrathecal infusion. After 14 months, bupivacaine treatment continued to control pain exacerbations. We conclude that CRPS patients benefit from early identification of the predominant underlying symptoms and a targeted treatment with moderately invasive techniques when less invasive techniques fail. Intrathecal bupivacaine, bupivacaine monotherapy, retrograde catheter, complex regional pain syndrome (CRPS), dual stimulation, dosal root ganglion (DRG) testing.

  18. Rainfall Characteristic on the Slopes of Mount Merapi Region (Empirical Formula, Duration, Distribution, And Critical Line Woro River

    Directory of Open Access Journals (Sweden)

    Pudak Juni Laksana

    2015-05-01

    Full Text Available Debris flow on the slopes of Mount Merapi area became a serious natural disasters because it has great destructive force and velocity. Rainfall with a certain intensity and duration is one component triggering debris flow. Rainfall has variability of the temporal and spatial characteristics influenced by various factors, such as topography and climate. Dharma (2012 suggested to define the characteristics of the intensity of rainfall using rainfall data with a shorter duration with statistical tests to establish the best empirical IDF formula.  This research was using of 30 minutes rainfall data for short duration (<3 hours and a spreadsheet software representing duration and distribution of the rainfall. The most appropriate rainfall intensity formula was done by the empirical IDF formula, i.e. Sherman, Kimijima, Hasper and Mononobe. Rainfall intensity analysis applied Frequency Analysis Software (based on Microsoft Excel. Debris flow occurrence was analyzed using MLIT for method A to establish standard rainfall index.  Sherman formula performed the best fit to the IDF characteristics of rainfall in the region of the slopes of Mount Merapi. Rainfall distribution pattern showed high intensity rainfall in the first hour and then decreased in the next hour which means distribution for the duration of 3 hours, 12%, 28%, 25%, 16%, 12%, and 7%, respectively with an interval of 30 minutes. Based on critical line, 5 mm of standard rainfall index was gained in the case of warning (R1 and 28 mm in the case of evacuation (R2.

  19. Observation of parametric instabilities in the quarter critical density region driven by the Nike KrF laser

    Energy Technology Data Exchange (ETDEWEB)

    Weaver, J. L.; Kehne, D.; Brown, C. M.; Obenschain, S. P.; Serlin, V.; Schmitt, A. J. [U.S. Naval Research Laboratory, Washington DC 20375 (United States); Oh, J.; Lehmberg, R. H.; Mclean, E.; Manka, C. [Research Support Instruments, Lanham, Maryland 20905 (United States); Phillips, L. [Alogus Research Corporation, McLean, Virginia 22101 (United States); Afeyan, B. [Polymath Research, Inc., Pleasanton, California 94566 (United States); Seely, J.; Feldman, U. [Berkeley Research Associates, Inc., Beltsville, Maryland 20705 (United States)

    2013-02-15

    The krypton-fluoride (KrF) laser is an attractive choice for inertial confinement fusion due to its combination of short wavelength ({lambda}=248 nm), large bandwidth (up to 3 THz), and superior beam smoothing by induced spatial incoherence. These qualities improve the overall hydrodynamics of directly driven pellet implosions and should allow use of increased laser intensity due to higher thresholds for laser plasma instabilities when compared to frequency tripled Nd:glass lasers ({lambda}=351 nm). Here, we report the first observations of the two-plasmon decay instability using a KrF laser. The experiments utilized the Nike laser facility to irradiate solid plastic planar targets over a range of pulse lengths (0.35 ns{<=}{tau}{<=}1.25 ns) and intensities (up to 2 Multiplication-Sign 10{sup 15} W/cm{sup 2}). Variation of the laser pulse created different combinations of electron temperature and electron density scale length. The observed onset of instability growth was consistent with the expected scaling that KrF lasers have a higher intensity threshold for instabilities in the quarter critical density region.

  20. Observation of parametric instabilities in the quarter critical density region driven by the Nike KrF laser

    International Nuclear Information System (INIS)

    Weaver, J. L.; Kehne, D.; Brown, C. M.; Obenschain, S. P.; Serlin, V.; Schmitt, A. J.; Oh, J.; Lehmberg, R. H.; Mclean, E.; Manka, C.; Phillips, L.; Afeyan, B.; Seely, J.; Feldman, U.

    2013-01-01

    The krypton-fluoride (KrF) laser is an attractive choice for inertial confinement fusion due to its combination of short wavelength (λ=248 nm), large bandwidth (up to 3 THz), and superior beam smoothing by induced spatial incoherence. These qualities improve the overall hydrodynamics of directly driven pellet implosions and should allow use of increased laser intensity due to higher thresholds for laser plasma instabilities when compared to frequency tripled Nd:glass lasers (λ=351 nm). Here, we report the first observations of the two-plasmon decay instability using a KrF laser. The experiments utilized the Nike laser facility to irradiate solid plastic planar targets over a range of pulse lengths (0.35 ns≤τ≤1.25 ns) and intensities (up to 2×10 15 W/cm 2 ). Variation of the laser pulse created different combinations of electron temperature and electron density scale length. The observed onset of instability growth was consistent with the expected scaling that KrF lasers have a higher intensity threshold for instabilities in the quarter critical density region.

  1. Faulting in the Yucca Mountain region: Critical review and analyses of tectonic data from the central Basin and Range

    International Nuclear Information System (INIS)

    Ferrill, D.A.; Stirewalt, G.L.; Henderson, D.B.; Stamatakos, J.; Morris, A.P.; Spivey, K.H.; Wernicke, B.P.

    1996-03-01

    Yucca Mountain, Nevada, has been proposed as the potential site for a high-level waste (HLW) repository. The tectonic setting of Yucca Mountain presents several potential hazards for a proposed repository, such as potential for earthquake seismicity, fault disruption, basaltic volcanism, magma channeling along pre-existing faults, and faults and fractures that may serve as barriers or conduits for groundwater flow. Characterization of geologic structures and tectonic processes will be necessary to assess compliance with regulatory requirements for the proposed high level waste repository. In this report, we specifically investigate fault slip, seismicity, contemporary stain, and fault-slip potential in the Yucca Mountain region with regard to Key Technical Uncertainties outlined in the License Application Review Plan (Sections 3.2.1.5 through 3.2.1.9 and 3.2.2.8). These investigations center on (i) alternative methods of determining the slip history of the Bare Mountain Fault, (ii) cluster analysis of historic earthquakes, (iii) crustal strain determinations from Global Positioning System measurements, and (iv) three-dimensional slip-tendency analysis. The goal of this work is to assess uncertainties associated with neotectonic data sets critical to the Nuclear Regulatory Commission and the Center for Nuclear Waste Regulatory Analyses' ability to provide prelicensing guidance and perform license application review with respect to the proposed HLW repository at Yucca Mountain

  2. Observation of parametric instabilities in the quarter critical density region driven by the Nike KrF laser

    Science.gov (United States)

    Weaver, J. L.; Oh, J.; Phillips, L.; Afeyan, B.; Seely, J.; Kehne, D.; Brown, C. M.; Obenschain, S. P.; Serlin, V.; Schmitt, A. J.; Feldman, U.; Lehmberg, R. H.; Mclean, E.; Manka, C.

    2013-02-01

    The krypton-fluoride (KrF) laser is an attractive choice for inertial confinement fusion due to its combination of short wavelength (λ =248 nm), large bandwidth (up to 3 THz), and superior beam smoothing by induced spatial incoherence. These qualities improve the overall hydrodynamics of directly driven pellet implosions and should allow use of increased laser intensity due to higher thresholds for laser plasma instabilities when compared to frequency tripled Nd:glass lasers (λ =351 nm). Here, we report the first observations of the two-plasmon decay instability using a KrF laser. The experiments utilized the Nike laser facility to irradiate solid plastic planar targets over a range of pulse lengths (0.35 ns≤τ≤1.25 ns) and intensities (up to 2×1015 W/cm2). Variation of the laser pulse created different combinations of electron temperature and electron density scale length. The observed onset of instability growth was consistent with the expected scaling that KrF lasers have a higher intensity threshold for instabilities in the quarter critical density region.

  3. Variation in critical care unit admission rates and outcomes for patients with acute coronary syndromes or heart failure among high- and low-volume cardiac hospitals.

    Science.gov (United States)

    van Diepen, Sean; Bakal, Jeffrey A; Lin, Meng; Kaul, Padma; McAlister, Finlay A; Ezekowitz, Justin A

    2015-02-27

    Little is known about cross-hospital differences in critical care units admission rates and related resource utilization and outcomes among patients hospitalized with acute coronary syndromes (ACS) or heart failure (HF). Using a population-based sample of 16,078 patients admitted to a critical care unit with a primary diagnosis of ACS (n=14,610) or HF (n=1467) between April 1, 2003 and March 31, 2013 in Alberta, Canada, we stratified hospitals into high (>250), medium (200 to 250), or low (<200) volume based on their annual volume of all ACS and HF hospitalization. The percentage of hospitalized patients admitted to critical care units varied across low, medium, and high-volume hospitals for both ACS and HF as follows: 77.9%, 81.3%, and 76.3% (P<0.001), and 18.0%, 16.3%, and 13.0% (P<0.001), respectively. Compared to low-volume units, critical care patients with ACS and HF admitted to high-volume hospitals had shorter mean critical care stays (56.6 versus 95.6 hours, P<0.001), more critical care procedures (1.9 versus 1.2 per patient, <0.001), and higher resource-intensive weighting (2.8 versus 1.5, P<0.001). No differences in in-hospital mortality (5.5% versus 6.2%, adjusted odds ratio 0.93; 95% CI, 0.61 to 1.41) were observed between high- and low-volume hospitals; however, 30-day cardiovascular readmissions (4.6% versus 6.8%, odds ratio 0.77; 95% CI, 0.60 to 0.99) and cardiovascular emergency-room visits (6.6% versus 9.5%, odds ratio 0.80; 95% CI, 0.69 to 0.94) were lower in high-volume compared to low-volume hospitals. Outcomes stratified by ACS or HF admission diagnosis were similar. Cardiac patients hospitalized in low-volume hospitals were more frequently admitted to critical care units and had longer hospitals stays despite lower resource-intensive weighting. These findings may provide opportunities to standardize critical care utilization for ACS and HF patients across high- and low-volume hospitals. © 2015 The Authors. Published on behalf of the American

  4. Screening of MITF and SOX10 regulatory regions in Waardenburg syndrome type 2.

    Directory of Open Access Journals (Sweden)

    Viviane Baral

    Full Text Available Waardenburg syndrome (WS is a rare auditory-pigmentary disorder that exhibits varying combinations of sensorineural hearing loss and pigmentation defects. Four subtypes are clinically defined based on the presence or absence of additional symptoms. WS type 2 (WS2 can result from mutations within the MITF or SOX10 genes; however, 70% of WS2 cases remain unexplained at the molecular level, suggesting that other genes might be involved and/or that mutations within the known genes escaped previous screenings. The recent identification of a deletion encompassing three of the SOX10 regulatory elements in a patient presenting with another WS subtype, WS4, defined by its association with Hirschsprung disease, led us to search for deletions and point mutations within the MITF and SOX10 regulatory elements in 28 yet unexplained WS2 cases. Two nucleotide variations were identified: one in close proximity to the MITF distal enhancer (MDE and one within the U1 SOX10 enhancer. Functional analyses argued against a pathogenic effect of these variations, suggesting that mutations within regulatory elements of WS genes are not a major cause of this neurocristopathy.

  5. Screening of MITF and SOX10 Regulatory Regions in Waardenburg Syndrome Type 2

    Science.gov (United States)

    Baral, Viviane; Chaoui, Asma; Watanabe, Yuli; Goossens, Michel; Attie-Bitach, Tania; Marlin, Sandrine; Pingault, Veronique; Bondurand, Nadege

    2012-01-01

    Waardenburg syndrome (WS) is a rare auditory-pigmentary disorder that exhibits varying combinations of sensorineural hearing loss and pigmentation defects. Four subtypes are clinically defined based on the presence or absence of additional symptoms. WS type 2 (WS2) can result from mutations within the MITF or SOX10 genes; however, 70% of WS2 cases remain unexplained at the molecular level, suggesting that other genes might be involved and/or that mutations within the known genes escaped previous screenings. The recent identification of a deletion encompassing three of the SOX10 regulatory elements in a patient presenting with another WS subtype, WS4, defined by its association with Hirschsprung disease, led us to search for deletions and point mutations within the MITF and SOX10 regulatory elements in 28 yet unexplained WS2 cases. Two nucleotide variations were identified: one in close proximity to the MITF distal enhancer (MDE) and one within the U1 SOX10 enhancer. Functional analyses argued against a pathogenic effect of these variations, suggesting that mutations within regulatory elements of WS genes are not a major cause of this neurocristopathy. PMID:22848661

  6. Hantavirus pulmonary syndrome and rodent reservoirs in the savanna-like biome of Brazil's southeastern region.

    Science.gov (United States)

    Limongi, J E; Oliveira, R C; Guterres, A; Costa Neto, S F; Fernandes, J; Vicente, L H B; Coelho, M G; Ramos, V N; Ferreira, M S; Bonvicino, C R; D'Andrea, P S; Lemos, E R S

    2016-04-01

    This paper describes the diversity of rodent fauna in an area endemic for hantavirus cardiopulmonary syndrome (HCPS) in Brazil, the population dynamics and the relationship of rodents with hantavirus in the Cerrado (savanna-like) biome. Additionally, an analysis is made of the partial S segment sequences of the hantaviruses obtained from serologically confirmed human HCPS cases and from rodent specimens. Rodents were collected during four campaigns. Human serum samples were collected from suspected cases of HCPS at hospitals in the state of Minas Gerais. The samples antibody-reactive by ELISA were processed by RT-PCR. The PCR product was amplified and sequenced. Hantavirus was detected only in Necromys lasiurus, the wild rodent species most prevalent in the Cerrado biome (min-max: 50-83·7%). All the six human serum samples were hantavirus seropositive and five showed amplified PCR products. The analysis of the nucleotide sequences showed the circulation of a single genotype, the Araraquara hantavirus. The environmental changes that have occurred in the Cerrado biome in recent decades have favoured N. lasiurus in interspecific competition of habitats, thus increasing the risk of contact between humans and rodent species infected with hantavirus. Our data corroborate the definition of N. lasiurus as the main hantavirus reservoir in the Cerrado biome.

  7. Association of polymorphisms of interleukin-18 gene promoter region with polycystic ovary syndrome in chinese population

    Directory of Open Access Journals (Sweden)

    Li Mei-zhi

    2010-10-01

    Full Text Available Abstract Background Recent research shows that polycystic ovary syndrome (PCOS may have an association with low-grade chronic inflammation, and that PCOS may induce an increase in serum interleukin-18 (IL-18 levels. Methods To investigate the polymorphisms of the IL-18 gene promoters with PCOS, two single nucleotide polymorphisms (SNPs in the promoter of the IL-18 gene (at positions -607C/A and -137G/C in 118 Chinese women with PCOS and 79 controls were evaluated using polymerase chain reaction (PCR. Results No significant differences were found in the genotype distribution, allele frequency and haplotype frequency between the PCOS and control groups. Further analysis demonstrated a relationship between IL-18 gene promoter polymorphisms and PCOS insulin resistance (IR. Regarding the -137 allele frequency, G and C allele frequencies were 93.5% and 6.5%, respectively, in the PCOS with IR patients; G and C allele frequencies were 85.4% and 14.6%, respectively, in PCOS patients without IR (chi2 = 3.601, P = 0.048. Conclusions The presence of a polymorphism in the IL-18 gene was found to have no correlation with the occurrence of PCOS. Carriage of the C allele at position -137 in the promoter of the IL-18 gene may play a protective role from the development of PCOS IR.

  8. Screening of MITF and SOX10 regulatory regions in Waardenburg syndrome type 2.

    Science.gov (United States)

    Baral, Viviane; Chaoui, Asma; Watanabe, Yuli; Goossens, Michel; Attie-Bitach, Tania; Marlin, Sandrine; Pingault, Veronique; Bondurand, Nadege

    2012-01-01

    Waardenburg syndrome (WS) is a rare auditory-pigmentary disorder that exhibits varying combinations of sensorineural hearing loss and pigmentation defects. Four subtypes are clinically defined based on the presence or absence of additional symptoms. WS type 2 (WS2) can result from mutations within the MITF or SOX10 genes; however, 70% of WS2 cases remain unexplained at the molecular level, suggesting that other genes might be involved and/or that mutations within the known genes escaped previous screenings. The recent identification of a deletion encompassing three of the SOX10 regulatory elements in a patient presenting with another WS subtype, WS4, defined by its association with Hirschsprung disease, led us to search for deletions and point mutations within the MITF and SOX10 regulatory elements in 28 yet unexplained WS2 cases. Two nucleotide variations were identified: one in close proximity to the MITF distal enhancer (MDE) and one within the U1 SOX10 enhancer. Functional analyses argued against a pathogenic effect of these variations, suggesting that mutations within regulatory elements of WS genes are not a major cause of this neurocristopathy.

  9. Regional Brain Glucose Hypometabolism in Young Women with Polycystic Ovary Syndrome: Possible Link to Mild Insulin Resistance.

    Science.gov (United States)

    Castellano, Christian-Alexandre; Baillargeon, Jean-Patrice; Nugent, Scott; Tremblay, Sébastien; Fortier, Mélanie; Imbeault, Hélène; Duval, Julie; Cunnane, Stephen C

    2015-01-01

    To investigate whether cerebral metabolic rate of glucose (CMRglu) is altered in normal weight young women with polycystic ovary syndrome (PCOS) who exhibit mild insulin resistance. Seven women with PCOS were compared to eleven healthy female controls of similar age, education and body mass index. Regional brain glucose uptake was quantified using FDG with dynamic positron emission tomography and magnetic resonance imaging, and its potential relationship with insulin resistance assessed using the updated homeostasis model assessment (HOMA2-IR). A battery of cognitive tests was administered to evaluate working memory, attention and executive function. The PCOS group had 10% higher fasting glucose and 40% higher HOMA2-IR (p ≤ 0.035) compared to the Controls. The PCOS group had 9-14% lower CMRglu in specific regions of the frontal, parietal and temporal cortices (p ≤ 0.018). A significant negative relation was found between the CMRglu and HOMA2-IR mainly in the frontal, parietal and temporal cortices as well as in the hippocampus and the amygdala (p ≤ 0.05). Globally, cognitive performance was normal in both groups but scores on the PASAT test of working memory tended to be low in the PCOS group. The PCOS group exhibited a pattern of low regional CMRglu that correlated inversely with HOMA2-IR in several brain regions and which resembled the pattern seen in aging and early Alzheimer's disease. These results suggest that a direct association between mild insulin resistance and brain glucose hypometabolism independent of overweight or obesity can exist in young adults in their 20s. Further investigation of the influence of insulin resistance on brain glucose metabolism and cognition in younger and middle-aged adults is warranted.

  10. Regional Brain Glucose Hypometabolism in Young Women with Polycystic Ovary Syndrome: Possible Link to Mild Insulin Resistance.

    Directory of Open Access Journals (Sweden)

    Christian-Alexandre Castellano

    Full Text Available To investigate whether cerebral metabolic rate of glucose (CMRglu is altered in normal weight young women with polycystic ovary syndrome (PCOS who exhibit mild insulin resistance.Seven women with PCOS were compared to eleven healthy female controls of similar age, education and body mass index. Regional brain glucose uptake was quantified using FDG with dynamic positron emission tomography and magnetic resonance imaging, and its potential relationship with insulin resistance assessed using the updated homeostasis model assessment (HOMA2-IR. A battery of cognitive tests was administered to evaluate working memory, attention and executive function.The PCOS group had 10% higher fasting glucose and 40% higher HOMA2-IR (p ≤ 0.035 compared to the Controls. The PCOS group had 9-14% lower CMRglu in specific regions of the frontal, parietal and temporal cortices (p ≤ 0.018. A significant negative relation was found between the CMRglu and HOMA2-IR mainly in the frontal, parietal and temporal cortices as well as in the hippocampus and the amygdala (p ≤ 0.05. Globally, cognitive performance was normal in both groups but scores on the PASAT test of working memory tended to be low in the PCOS group.The PCOS group exhibited a pattern of low regional CMRglu that correlated inversely with HOMA2-IR in several brain regions and which resembled the pattern seen in aging and early Alzheimer's disease. These results suggest that a direct association between mild insulin resistance and brain glucose hypometabolism independent of overweight or obesity can exist in young adults in their 20s. Further investigation of the influence of insulin resistance on brain glucose metabolism and cognition in younger and middle-aged adults is warranted.

  11. Treatment of Patients With Complex Regional Pain Syndrome Type I With Mannitol: A Prospective, Randomized, Placebo-Controlled, Double-Blinded Study

    NARCIS (Netherlands)

    Perez, R.S.G.M.; Pragt, E.; Geurts, J.J.G.; Zuurmond, W.W.A.; Patijn, J.; van Kleef, M.

    2008-01-01

    To assess the effects of intravenous administration of the free radical scavenger mannitol 10% on complaints associated with complex regional pain syndrome Type I (CRPS I), a randomized, placebo-controlled, double-blinded trial was performed. Forty-one CRPS I patients according to the Bruehl et al

  12. Modified Graded Motor Imagery for Complex Regional Pain Syndrome Type 1 of the Upper Extremity in the Acute Phase: A Patient Series

    Science.gov (United States)

    Lagueux, Emilie; Charest, Joelle; Lefrancois-Caron, Eve; Mauger, Marie-Eve; Mercier, Emilie; Savard, Kim; Tousignant-Laflamme, Yannick

    2012-01-01

    Complex regional pain syndrome (CRPS) is a pathologic condition in which the painful experience is disproportionate in time and intensity in comparison with the inciting event. At present, the pathophysiology of CRPS is not well understood. Several studies have indicated that cortical reorganization plays a role in the persistence of the symptoms.…

  13. Oxidative Stress in Complex Regional Pain Syndrome (CRPS): No Systemically Elevated Levels of Malondialdehyde, F2-Isoprostanes and 8OHdG in a Selected Sample of Patients

    NARCIS (Netherlands)

    Fischer, S.G.L.; Perez, R.S.G.M.; Nouta, J.; Zuurmond, W.W.A.; Scheffer, P.G.

    2013-01-01

    Exaggerated inflammation and oxidative stress are involved in the pathogenesis of Complex Regional Pain Syndrome (CRPS). However, studies assessing markers for oxidative stress in CRPS patients are limited. In this study, markers for lipid peroxidation (malondialdehyde and F2-isoprostanes) and DNA

  14. Deletions and rearrangements of the H19/IGF2 enhancer region in patients with Silver-Russell syndrome and growth retardation

    DEFF Research Database (Denmark)

    Grønskov, Karen; Poole, Rebecca L; Hahnemann, Johanne M D

    2011-01-01

    Silver-Russell syndrome (SRS) is characterised by prenatal and postnatal growth retardation, dysmorphic facial features, and body asymmetry. In 35-60% of SRS cases the paternally methylated imprinting control region (ICR) upstream of the H19 gene (H19-ICR) is hypomethylated, leading to downregula...

  15. Indel-II region deletion sizes in the white spot syndrome virus genome correlate with shrimp disease outbreaks in southern Vietnam

    NARCIS (Netherlands)

    Tran Thi Tuyet, H.; Zwart, M.P.; Phuong, N.T.; Oanh, D.T.H.; Jong, de M.C.M.; Vlak, J.M.

    2012-01-01

    Sequence comparisons of the genomes of white spot syndrome virus (WSSV) strains have identified regions containing variable-length insertions/deletions (i.e. indels). Indel-I and Indel-II, positioned between open reading frames (ORFs) 14/15 and 23/24, respectively, are the largest and the most

  16. Complex regional pain syndrome type I : Use of the International Association for the Study of Pain diagnostic criteria defined in 1994

    NARCIS (Netherlands)

    Reinders, M.F.; Geertzen, J.H.B.; Dijkstra, P.U.

    2002-01-01

    Objectives: The objective was to assess the reported use in recent publications of the diagnostic criteria for complex regional pain syndrome type I (CRPS 1) proposed by the International Association for the Study of Pain (IASP) in 1994. Methods: A literature search of MEDLINE (January 1996 to July

  17. Development of a symptoms questionnaire for complex regional pain syndrome and potentially related illnesses: the Trauma Related Neuronal Dysfunction Symptoms Inventory

    NARCIS (Netherlands)

    Collins, S.; van Hilten, J.J.; Marinus, J.J.; Zuurmond, W.W.A.; de Lange, J.J.; Perez, R.S.G.M.

    2008-01-01

    Collins S, van Hilten JJ, Marinus J, Zuurmond WW, de Lange JJ, Perez RS. Development of a symptoms questionnaire for complex regional pain syndrome and potentially related illnesses: the Trauma Related Neuronal Dysfunction Symptoms Inventory. Objective: To develop a questionnaire to evaluate

  18. Management of infections in critically ill returning travellers in the intensive care unit—II: clinical syndromes and special considerations in immunocompromised patients

    Directory of Open Access Journals (Sweden)

    Jordi Rello

    2016-07-01

    Full Text Available This position paper is the second ESCMID Consensus Document on this subject and aims to provide intensivists, infectious disease specialists, and emergency physicians with a standardized approach to the management of serious travel-related infections in the intensive care unit (ICU or the emergency department. This document is a cooperative effort between members of two European Society of Clinical Microbiology and Infectious Diseases (ESCMID study groups and was coordinated by Hakan Leblebicioglu and Jordi Rello for ESGITM (ESCMID Study Group for Infections in Travellers and Migrants and ESGCIP (ESCMID Study Group for Infections in Critically Ill Patients, respectively. A relevant expert on the subject of each section prepared the first draft which was then edited and approved by additional members from both ESCMID study groups. This article summarizes considerations regarding clinical syndromes requiring ICU admission in travellers, covering immunocompromised patients.

  19. Inflammatory peeling skin syndrome caused by homozygous genomic deletion in the PSORS1 region encompassing the CDSN gene.

    Science.gov (United States)

    Ishida-Yamamoto, Akemi; Furio, Laetitia; Igawa, Satomi; Honma, Masaru; Tron, Elodie; Malan, Valerie; Murakami, Masamoto; Hovnanian, Alain

    2014-01-01

    Peeling skin syndrome (PSS) type B is a rare recessive genodermatosis characterized by lifelong widespread, reddish peeling of the skin with pruritus. The disease is caused by small-scale mutations in the Corneodesmosin gene (CDSN) leading to premature termination codons. We report for the first time a Japanese case resulting from complete deletion of CDSN. Corneodesmosin was undetectable in the epidermis, and CDSN was unamplifiable by PCR. QMPSF analysis demonstrated deletion of CDSN exons inherited from each parent. Deletion mapping using microsatellite haplotyping, CGH array and PCR analysis established that the genomic deletion spanned 49-72 kb between HCG22 and TCF19, removing CDSN as well as five other genes within the psoriasis susceptibility region 1 (PSORS1) on 6p21.33. This observation widens the spectrum of molecular defects underlying PSS type B and shows that loss of these five genes from the PSORS1 region does not result in an additional cutaneous phenotype. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Knowledge translation lessons from an audit of Aboriginal Australians with acute coronary syndrome presenting to a regional hospital.

    Science.gov (United States)

    Haynes, Emma; Hohnen, Harry; Katzenellenbogen, Judith M; Scalley, Benjamin D; Thompson, Sandra C

    2016-01-01

    Translation of evidence into practice by health systems can be slow and incomplete and may disproportionately impact disadvantaged populations. Coronary heart disease is the leading cause of death among Aboriginal Australians. Timely access to effective medical care for acute coronary syndrome substantially improves survival. A quality-of-care audit conducted at a regional Western Australian hospital in 2011-2012 compared the Emergency Department management of Aboriginal and non-Aboriginal acute coronary syndrome patients. This audit is used as a case study of translating knowledge processes in order to identify the factors that support equity-oriented knowledge translation. In-depth interviews were conducted with a purposive sample of the audit team and further key stakeholders with interest/experience in knowledge translation in the context of Aboriginal health. Interviews were analysed for alignment of the knowledge translation process with the thematic steps outlined in Tugwell's cascade for equity-oriented knowledge translation framework. In preparing the audit, groundwork helped shape management support to ensure receptivity to targeting Aboriginal cardiovascular outcomes. Reporting of audit findings and resulting advocacy were undertaken by the audit team with awareness of the institutional hierarchy, appropriate timing, personal relationships and recognising the importance of tailoring messages to specific audiences. These strategies were also acknowledged as important in the key stakeholder interviews. A follow-up audit documented a general improvement in treatment guideline adherence and a reduction in treatment inequalities for Aboriginal presentations. As well as identifying outcomes such as practice changes, a useful evaluation increases understanding of why and how an intervention worked. Case studies such as this enrich our understanding of the complex human factors, including individual attributes, experiences and relationships and systemic factors

  1. Knowledge translation lessons from an audit of Aboriginal Australians with acute coronary syndrome presenting to a regional hospital

    Directory of Open Access Journals (Sweden)

    Emma Haynes

    2016-07-01

    Full Text Available Objective: Translation of evidence into practice by health systems can be slow and incomplete and may disproportionately impact disadvantaged populations. Coronary heart disease is the leading cause of death among Aboriginal Australians. Timely access to effective medical care for acute coronary syndrome substantially improves survival. A quality-of-care audit conducted at a regional Western Australian hospital in 2011–2012 compared the Emergency Department management of Aboriginal and non-Aboriginal acute coronary syndrome patients. This audit is used as a case study of translating knowledge processes in order to identify the factors that support equity-oriented knowledge translation. Methods: In-depth interviews were conducted with a purposive sample of the audit team and further key stakeholders with interest/experience in knowledge translation in the context of Aboriginal health. Interviews were analysed for alignment of the knowledge translation process with the thematic steps outlined in Tugwell’s cascade for equity-oriented knowledge translation framework. Results: In preparing the audit, groundwork helped shape management support to ensure receptivity to targeting Aboriginal cardiovascular outcomes. Reporting of audit findings and resulting advocacy were undertaken by the audit team with awareness of the institutional hierarchy, appropriate timing, personal relationships and recognising the importance of tailoring messages to specific audiences. These strategies were also acknowledged as important in the key stakeholder interviews. A follow-up audit documented a general improvement in treatment guideline adherence and a reduction in treatment inequalities for Aboriginal presentations. Conclusion: As well as identifying outcomes such as practice changes, a useful evaluation increases understanding of why and how an intervention worked. Case studies such as this enrich our understanding of the complex human factors, including

  2. Velo-Cardio-Facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region

    Energy Technology Data Exchange (ETDEWEB)

    Nickel, R.E.; Pillers, D.M.; Merkens, M.; Magenis, R.E.; Zonana, J. [Oregon Health Sciences Univ., Portland, OR (United States); Driscoll, D.A.; Emanuel, B.S. [Univ. of Pennsylvania Medical Center, Philadelphia, PA (United States)

    1994-10-01

    Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both have bifid uvula. The third child had DiGeorge sequence (DGS). The association of NTDs with 22q11 deletion has not been reported previously. An accurate diagnosis of the 22q11 deletion is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions. 31 refs., 3 figs.

  3. Two-phase flow characteristic of inverted bubbly, slug, and annular flow in post-critical heat flux region

    International Nuclear Information System (INIS)

    Ishii, M.; Denten, J.P.

    1989-01-01

    Inverted annular flow can be visualized as a liquid jet-like core surrounded by a vapor annulus. While many analytical and experimental studies of heat transfer in this regime have been performed, there is very little understanding of the basic hydrodynamics of the post-critical heat flux (CHF) flow field. However, a recent experimental study was done that was able to successfully investigate the effects of various steady-state inlet flow parameters on the post-CHF hydrodynamics of the film boiling of a single phase liquid jet. This study was carried out by means of a visual photographic analysis of an idealized single phase core inverted annular flow initial geometry (single phase liquid jet core surrounded by a coaxial annulus of gas). In order to extend this study, a subsequent flow visualization of an idealized two-phase core inverted annular flow geometry (two-phase central jet core, surrounded by a coaxial annulus of gas) was carried out. The objective of this second experimental study was to investigate the effect of steady-state inlet, pre-CHF two-phase jet core parameters on the hydrodynamics of the post-CHF flow field. In actual film boiling situations, two-phase flows with net positive qualities at the CHF point are encountered. Thus, the focus of the present experimental study was on the inverted bubbly, slug, and annular flow fields in the post dryout film boiling region. Observed post dryout hydrodynamic behavior is reported. A correlation for the axial extent of the transition flow pattern between inverted annular and dispersed droplet flow (the agitated regime) is developed. It is shown to depend strongly on inlet jet core parameters and jet void fraction at the dryout point

  4. Evaluation of low level laser and interferential current in the therapy of complex regional pain syndrome by infrared thermographic camera

    Directory of Open Access Journals (Sweden)

    Kocić Mirjana

    2010-01-01

    Full Text Available Background/Aim. Complex regional pain syndrome type I (CRPS I is characterized by continuous regional pain, disproportional according to duration and intensity and to the sort of trauma or other lesion it was caused by. The aim of the study was to evaluate and compare, by using thermovison, the effects of low level laser therapy and therapy with interferential current in treatment of CRPS I. Methods. The prospective randomized controlled clinical study included 45 patients with unilateral CRPS I, after a fracture of the distal end of the radius, of the tibia and/or the fibula, treated in the Clinical Centre in Nis from 2004 to 2007. The group A consisted of 20 patients treated by low level laser therapy and kinesy-therapy, while the patients in the group B (n = 25 were treated by interferential current and kinesy-therapy. The regions of interest were filmed by a thermovision camera on both sides, before and after the 20 therapeutic procedures had been applied. Afterwards, the quantitative analysis and the comparing of thermograms taken before and after the applied therapy were performed. Results. There was statistically significant decrease of the mean maximum temperature difference between the injured and the contralateral extremity after the therapy in comparison to the status before the therapy, with the patients of the group A (p < 0.001 as well as those of the group B (p < 0.001. The decrease was statistically significantly higher in the group A than in the group B (p < 0.05. Conclusions. By the use of the infrared thermovision we showed that in the treatment of CRPS I both physical medicine methods were effective, but the effectiveness of laser therapy was statistically significantly higher compared to that of the interferential current therapy.

  5. Bisphosphonates for treatment of Complex Regional Pain Syndrome type 1: A systematic literature review and meta-analysis of randomized controlled trials versus placebo.

    Science.gov (United States)

    Chevreau, Maxime; Romand, Xavier; Gaudin, Philippe; Juvin, Robert; Baillet, Athan

    2017-07-01

    Complex Regional Pain Syndrome Type 1 is a severely disabling pain syndrome with no definite established treatment. We have performed a systematic literature review and meta-analysis of all randomized controlled trials to assess the benefit of bisphosphonates on pain and function in patients with Complex Regional Pain Syndrome Type 1. A systematic literature search was performed in the Medline, Embase and Cochrane databases. Two authors selected independently blinded randomized trials comparing bisphosphonates to placebo on short-term (J30 to J40) and medium term pain (M2-M3), safety and function in patients with CRPS 1. The methodological quality of the studies was analyzed. Data were aggregated using the method of the inverse of the variance. 258 articles were identified. Four trials of moderate to good quality comprising 181 patients (90 in the bisphosphonate group and 91 in the placebo group) were included in this meta-analysis. Short-term pain Visual Analog Scale was significantly lower in the bisphosphonate group versus the placebo group (SMD=-2.6, 95%CI [-1.8, -3.4], Ppain (SMD=-2.5, 95%CI [-1.4, -3.6], Ppain in patients with Complex Regional Pain Syndrome type 1. Other studies are needed to determine their effectiveness. Copyright © 2017. Published by Elsevier SAS.

  6. Molecular cytogenetic analysis of Inv Dup(15) chromosomes, using probes specific for the Pradar-Willi/Angelman syndrome region: Clinical implications

    Energy Technology Data Exchange (ETDEWEB)

    Leana-Cox, J. (Univ. of Maryland School of Medicine, Baltimore, MD (United States)); Jenkins, L. (Kaiser Permanente Medical Group, San Jose, CA (United States)); Palmer, C.G.; Plattner, R. (Indiana School of Medicine, Indianapolis, IN (United States)); Sheppard, L. (Palo Verde Laboratory, Inc., Chandler, AZ (United States)); Flejter, W.L. (Univ. of Michigan, Ann Arbor, MI (United States)); Zackowski, J. (Univ. of Florida Health Science Center, Gainsville, FL (United States)); Tsien, F. (Tulane Univ. School of Medicine, New Orleans, LA (United States)); Schwartz, S. (Case Western Reserve Univ., Cleveland, OH (United States))

    1994-05-01

    Twenty-seven cases of inverted duplications of chromosome 15 (inv dup[15]) were investigated by FISH with two DNA probes specific for the Prader-Willi syndrome/Angelman syndrome (PWS/AS) region on proximal 15q. Sixteen of the marker chromosomes displayed two copies of each probe, while in the remaining 11 markers no hybridization was observed. A significant association was found between the presence of this region and an abnormal phenotype (P<.01). This is the largest study to date of inv dup(15) chromosomes, that uses molecular cytogenetic methods and is the first to report a significant association between the presence of a specific chromosomal region in such markers and an abnormal phenotype. 30 refs., 1 fig., 4 tabs.

  7. Emotion Potentiated Startle in Fragile X Syndrome

    Science.gov (United States)

    Ballinger, Elizabeth C.; Cordeiro, Lisa; Chavez, Alyssa D.; Hagerman, Randi J.; Hessl, David

    2014-01-01

    Social avoidance and anxiety are prevalent in fragile X syndrome (FXS) and are potentially mediated by the amygdala, a brain region critical for social behavior. Unfortunately, functional brain resonance imaging investigation of the amygdala in FXS is limited by the difficulties experienced by intellectually impaired and anxious participants. We…

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

    Directory of Open Access Journals (Sweden)

    Johnston Jennifer

    2011-07-01

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

  9. Contribution of regional cerebral blood flow changes on the evaluation of neuropsychological impairment in patients with Tourette syndrome

    International Nuclear Information System (INIS)

    Borbely, K.; Kadar, K.; Nagy, D.; Szlobodnyik, C.S.; Gadoros, J.; Radics, E.; Rozsa, S.

    2002-01-01

    Aims: The literature to date has suggested that intellectual ability is normally distributed in Tourette syndrome (TS). However, patients with TS have significant discrepancies between their verbal and non verbal abilities. Specific cognitive deficits in TS include visuomotor integration problems, impaired fine motor skill, and executive dysfunction. The presence of a learning disability or cognitive deficit may lead to a greater obstacle for patients with TS than the tic disorder itself. It would be important that patients with TS will be evaluated to having neuropsychological difficulties as soon as possible. The aim of this study was to evaluate the correlation between the clinical severity and neuropsychological impairment (especially memory deficit) of patients with TS and rCBF abnormalities. Methods: The assessment included neurological, psychiatrical examination (Yale Scale [YS], staging of the severity of disease), CT/MRI, and a specific test of explicit and implicit memory (Probabilistic Classification Learning test, PCL). 99m Tc-HMPAO-SPECT studies were carried out with a standard technique for each patient. The data were analyzed visually and by a special ROIs program. RCBF data were correlated to the results of the PCL test and the YS. Results: The SPECT measurements showed significant (p 40 in the temporal medial and lateral regions, and also in the cerebellum, compared to the patients with YS<40. Also, an increase of rCBF was observed in the left striatal, temporal medial and lateral regions, compared to the contralateral homologous regions (p<0.05). The YS (disease severity) demonstrated significant (p<0.05) and strong correlation with the rCBF in the right temporal region (r= -0.83) and in the thalamus (r=-0.69). The performance in the explicit memory test showed significant (p<0.05) and strong correlation with the perfusion in both temporopolar (r=0.9) and left temporomedial region (r=0.92). Conclusion: RCBF SPECT proved to be concordant with

  10. [Epizootology of hemorrhagic fever with renal syndrome in the Central Chernozem Region].

    Science.gov (United States)

    Balakirev, A E; Bashkirtsev, V N; Sedova, N S; Okulova, N M; Trankvilevskiĭ, D V; Sikora, I V; Frolova, S M; Luz'ianov, E N; Shinkorenko, N N; Sapel'nikov, A F; Tkachenko, E A

    2006-01-01

    A total of 5149 small mammals belonging to 16 species were collected from the Lipetsk, Voronezh, and Belgorod regions (40 administrative districts) in 2003-2004 and examined by ELISA and IFA to detect hantavirus antigen and antibodies in the lung tissues. Hantavirus circulation was revealed in 13 species, the highest hantavirus activity being detected in field (Apodemus agrarius) and small wood (A. (S) uralensis) mice (Dobrava-Belgrad virus), bank (Clethrionomis glareolus) (Puumala virus) and common (Microtus arvalis) (Tula virus) voles. These species were frequently found to have their untypical hantaviruses, which was most commonly observed in small wood mice. It is suggested that the small wood mouse is likely to take a certain part in maintaining the circulation of Dobrava-Belgrad virus.

  11. Early Retinal Defects in Fmr1−/y Mice: Toward a Critical Role of Visual Dys-Sensitivity in the Fragile X Syndrome Phenotype?

    Directory of Open Access Journals (Sweden)

    Olivier Perche

    2018-04-01

    Full Text Available Fragile X Syndrome (FXS is caused by a deficiency in Fragile X Mental Retardation Protein (FMRP leading to global sensorial abnormalities, among which visual defects represent a critical part. These visual defects are associated with cerebral neuron immaturity especially in the primary visual cortex. However, we recently demonstrated that retinas of adult Fmr1−/y mice, the FXS murine model, present molecular, cellular and functional alterations. However, no data are currently available on the evolution pattern of such defects. As retinal stimulation through Eye Opening (EO is a crucial signal for the cerebral visual system maturation, we questioned the precocity of molecular and functional retinal phenotype. To answer this question, we studied the retinal molecular phenotype of Fmr1−/y mice before EO until adult age and the consequences of the retinal loss of Fmrp on retinal function in young and adult mice. We showed that retinal molecular defects are present before EO and remain stable at adult age, leading to electrophysiological impairments without any underlying structural changes. We underlined that loss of Fmrp leads to a wide range of defects in the retina, settled even before EO. Our work demonstrates a critical role of the sensorial dysfunction in the Fmr1−/y mice overall phenotype, and provides evidence that altered peripheral perception is a component of the sensory processing defect in FXS conditions.

  12. Early Retinal Defects in Fmr1-/y Mice: Toward a Critical Role of Visual Dys-Sensitivity in the Fragile X Syndrome Phenotype?

    Science.gov (United States)

    Perche, Olivier; Felgerolle, Chloé; Ardourel, Maryvonne; Bazinet, Audrey; Pâris, Arnaud; Rossignol, Rafaëlle; Meyer-Dilhet, Géraldine; Mausset-Bonnefont, Anne-Laure; Hébert, Betty; Laurenceau, David; Montécot-Dubourg, Céline; Menuet, Arnaud; Bizot, Jean-Charles; Pichon, Jacques; Ranchon-Cole, Isabelle; Briault, Sylvain

    2018-01-01

    Fragile X Syndrome (FXS) is caused by a deficiency in Fragile X Mental Retardation Protein (FMRP) leading to global sensorial abnormalities, among which visual defects represent a critical part. These visual defects are associated with cerebral neuron immaturity especially in the primary visual cortex. However, we recently demonstrated that retinas of adult Fmr1 -/y mice, the FXS murine model, present molecular, cellular and functional alterations. However, no data are currently available on the evolution pattern of such defects. As retinal stimulation through Eye Opening (EO) is a crucial signal for the cerebral visual system maturation, we questioned the precocity of molecular and functional retinal phenotype. To answer this question, we studied the retinal molecular phenotype of Fmr1 -/y mice before EO until adult age and the consequences of the retinal loss of Fmrp on retinal function in young and adult mice. We showed that retinal molecular defects are present before EO and remain stable at adult age, leading to electrophysiological impairments without any underlying structural changes. We underlined that loss of Fmrp leads to a wide range of defects in the retina, settled even before EO. Our work demonstrates a critical role of the sensorial dysfunction in the Fmr1 -/y mice overall phenotype, and provides evidence that altered peripheral perception is a component of the sensory processing defect in FXS conditions.

  13. Early Retinal Defects in Fmr1−/y Mice: Toward a Critical Role of Visual Dys-Sensitivity in the Fragile X Syndrome Phenotype?

    Science.gov (United States)

    Perche, Olivier; Felgerolle, Chloé; Ardourel, Maryvonne; Bazinet, Audrey; Pâris, Arnaud; Rossignol, Rafaëlle; Meyer-Dilhet, Géraldine; Mausset-Bonnefont, Anne-Laure; Hébert, Betty; Laurenceau, David; Montécot-Dubourg, Céline; Menuet, Arnaud; Bizot, Jean-Charles; Pichon, Jacques; Ranchon-Cole, Isabelle; Briault, Sylvain

    2018-01-01

    Fragile X Syndrome (FXS) is caused by a deficiency in Fragile X Mental Retardation Protein (FMRP) leading to global sensorial abnormalities, among which visual defects represent a critical part. These visual defects are associated with cerebral neuron immaturity especially in the primary visual cortex. However, we recently demonstrated that retinas of adult Fmr1−/y mice, the FXS murine model, present molecular, cellular and functional alterations. However, no data are currently available on the evolution pattern of such defects. As retinal stimulation through Eye Opening (EO) is a crucial signal for the cerebral visual system maturation, we questioned the precocity of molecular and functional retinal phenotype. To answer this question, we studied the retinal molecular phenotype of Fmr1−/y mice before EO until adult age and the consequences of the retinal loss of Fmrp on retinal function in young and adult mice. We showed that retinal molecular defects are present before EO and remain stable at adult age, leading to electrophysiological impairments without any underlying structural changes. We underlined that loss of Fmrp leads to a wide range of defects in the retina, settled even before EO. Our work demonstrates a critical role of the sensorial dysfunction in the Fmr1−/y mice overall phenotype, and provides evidence that altered peripheral perception is a component of the sensory processing defect in FXS conditions. PMID:29681800

  14. Self-critical perfectionism and its relationship to fatigue and pain in the daily flow of life in patients with chronic fatigue syndrome.

    Science.gov (United States)

    Kempke, S; Luyten, P; Claes, S; Goossens, L; Bekaert, P; Van Wambeke, P; Van Houdenhove, B

    2013-05-01

    Research suggests that the personality factor of self-critical or maladaptive perfectionism may be implicated in chronic fatigue syndrome (CFS). However, it is not clear whether self-critical perfectionism (SCP) also predicts daily symptoms in CFS. Method In the present study we investigated whether SCP predicted fatigue and pain over a 14-day period in a sample of 90 CFS patients using a diary method approach. After completing the Depressive Experiences Questionnaire (DEQ) as a measure of SCP, patients were asked each day for 14 days to complete Visual Analogue Scales (VAS) of fatigue, pain and severity of depression. Data were analysed using multilevel analysis. The results from unconditional models revealed considerable fluctuations in fatigue over the 14 days, suggesting strong temporal variability in fatigue. By contrast, pain was relatively stable over time but showed significant inter-individual differences. Congruent with expectations, fixed-effect models showed that SCP was prospectively associated with higher daily fatigue and pain levels over the 14-day period, even after controlling for levels of depression. This is the first study to show that SCP predicts both fatigue and pain symptoms in CFS in the daily course of life. Hence, therapeutic interventions aimed at targeting SCP should be considered in the treatment of CFS patients with such features.

  15. Canine gastric dilatation/volvulus syndrome in a veterinary critical care unit: 295 cases (1986-1992).

    Science.gov (United States)

    Brockman, D J; Washabau, R J; Drobatz, K J

    1995-08-15

    Two hundred ninety-five case records were included in an analysis of dogs treated by a standardized protocol for gastric dilatation/volvulus syndrome between 1986 and 1992. A breed predisposition was demonstrated for Great Danes, German Shepherd Dogs, large mixed-breed dogs, and Standard Poodles. One hundred and ninety-three dogs had gastric dilatation and volvulus (GDV) confirmed at surgery, 66 had simple gastric dilatation (GD), and 36 others had gastric dilatation but volvulus could not be proved or disproved (GD +/- V). Among dogs with GDV, the fatality rate was 15% (29/193). Twenty-six (13.5%) dogs with GDV underwent partial gastrectomy, and 8 (31%) died or were subsequently euthanatized. In comparing the group of dogs with GDV that survived to those that died, there were no statistical differences in the age of dog, time between onset of clinical signs and admission, time from admission to surgery, or duration of anesthesia. Cardiac arrhythmias were detected in 40% (78/193) of the dogs with GDV. There also was no statistical correlation between development of a cardiac arrhythmia and outcome in dogs with GDV. The causes of death in dogs with GDV were multiple and varied; presumed gastric necrosis was a common reason for intraoperative euthanasia (11 dogs). Among dogs with GD or GD +/- V, the fatality rate was 0.9% (1/102).

  16. Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with down syndrome

    NARCIS (Netherlands)

    Blom, Nico A.; Ottenkamp, Jaap; Wenink, Arnold G. C.; Gittenberger-de Groot, Adriana C.

    2003-01-01

    Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic

  17. National and Regional Representativeness of Hospital Emergency Department Visit Data in the National Syndromic Surveillance Program, United States, 2014

    Science.gov (United States)

    Coates, Ralph J.; Pérez, Alejandro; Baer, Atar; Zhou, Hong; English, Roseanne; Coletta, Michael; Dey, Achintya

    2016-01-01

    Objective We examined the representativeness of the nonfederal hospital emergency department (ED) visit data in the National Syndromic Surveillance Program (NSSP). Methods We used the 2012 American Hospital Association Annual Survey Database, other databases, and information from state and local health departments participating in the NSSP about which hospitals submitted data to the NSSP in October 2014. We compared ED visits for hospitals submitting 15 data with all ED visits in all 50 states and Washington, DC. Results Approximately 60.4 million of 134.6 million ED visits nationwide (~45%) were reported to have been submitted to the NSSP. ED visits in 5 of 10 regions and the majority of the states were substantially underrepresented in the NSSP. The NSSP ED visits were similar to national ED visits in terms of many of the characteristics of hospitals and their service areas. However, visits in hospitals with the fewest annual ED visits, in rural trauma centers, and in hospitals serving populations with high percentages of Hispanics and Asians were underrepresented. Conclusions NSSP nonfederal hospital ED visit data were representative for many hospital characteristics and in some geographic areas but were not very representative nationally and in many locations. Representativeness could be improved by increasing participation in more states and among specific types of hospitals. PMID:26883318

  18. Usefulness of asymmetry score on quantitative three-phase bone scintigraphy in the evaluation of complex regional pain syndrome

    International Nuclear Information System (INIS)

    Sampath, Santhosh; Mittal, Bhagwant Rai; Arun, Sasikumar; Sood, Ashwani; Bhattacharya, Anish; Sharma, Aman

    2013-01-01

    Complex regional pain syndrome (CRPS) is primarily a clinical diagnosis. Diagnostic imaging in CRPS can be used, especially to exclude other disorders. The sensitivity and specificity of three phase bone scintigraphy (TPBS) for the diagnosis of CRPS is variable throughout the literature. To establish a simple and effective quantitative approach to help in the diagnosis of CRPS by TPBS. TPBS done in patients (n = 68) with suspected CRPS was analyzed retrospectively. They were classified into bone scan positive group (BSP), bone scan negative group (BSN) and non-CRPS group based on diffusely increased periarticular uptake, symmetrical uptake, and focal uptake respectively. Asymmetry score (AS) was also measured between the affected and unaffected side. 16 patients showed focal uptake, 37 were in BSP group with mean AS score of 1.57 ± 0.5 and 15 were in BSN group with mean AS score of 1.01 ± 0.05. The mean AS was significantly different (P < 0.0001). AS of 1.06 had sensitivity and specificity of 96.43% and 100% respectively (P = 0.0001). There was a trend of negative correlation between the AS and the duration, r = −0.21; however, it was not statistically significant (P = 0.28). TPBS should be considered in the evaluation of CRPS to rule out patients who have focal involvement, not diagnostic of CRPS (~24% in this study). Quantitative AS of 1.06 can be included to support visual interpretation in the delayed phase

  19. Enhanced pain and autonomic responses to ambiguous visual stimuli in chronic Complex Regional Pain Syndrome (CRPS) type I.

    Science.gov (United States)

    Cohen, H E; Hall, J; Harris, N; McCabe, C S; Blake, D R; Jänig, W

    2012-02-01

    Cortical reorganisation of sensory, motor and autonomic systems can lead to dysfunctional central integrative control. This may contribute to signs and symptoms of Complex Regional Pain Syndrome (CRPS), including pain. It has been hypothesised that central neuroplastic changes may cause afferent sensory feedback conflicts and produce pain. We investigated autonomic responses produced by ambiguous visual stimuli (AVS) in CRPS, and their relationship to pain. Thirty CRPS patients with upper limb involvement and 30 age and sex matched healthy controls had sympathetic autonomic function assessed using laser Doppler flowmetry of the finger pulp at baseline and while viewing a control figure or AVS. Compared to controls, there were diminished vasoconstrictor responses and a significant difference in the ratio of response between affected and unaffected limbs (symmetry ratio) to a deep breath and viewing AVS. While viewing visual stimuli, 33.5% of patients had asymmetric vasomotor responses and all healthy controls had a homologous symmetric pattern of response. Nineteen (61%) CRPS patients had enhanced pain within seconds of viewing the AVS. All the asymmetric vasomotor responses were in this group, and were not predictable from baseline autonomic function. Ten patients had accompanying dystonic reactions in their affected limb: 50% were in the asymmetric sub-group. In conclusion, there is a group of CRPS patients that demonstrate abnormal pain networks interacting with central somatomotor and autonomic integrational pathways. © 2011 European Federation of International Association for the Study of Pain Chapters.

  20. Antiallodynic Effects of Bee Venom in an Animal Model of Complex Regional Pain Syndrome Type 1 (CRPS-I).

    Science.gov (United States)

    Lee, Sung Hyun; Lee, Jae Min; Kim, Yun Hong; Choi, Jung Hyun; Jeon, Seung Hwan; Kim, Dong Kyu; Jeong, Hyeon Do; Lee, You Jung; Park, Hue Jung

    2017-09-15

    Neuropathic pain in a chronic post-ischaemic pain (CPIP) model mimics the symptoms of complex regional pain syndrome type I (CRPS I). The administration of bee venom (BV) has been utilized in Eastern medicine to treat chronic inflammatory diseases accompanying pain. However, the analgesic effect of BV in a CPIP model remains unknown. The application of a tight-fitting O-ring around the left ankle for a period of 3 h generated CPIP in C57/Bl6 male adult mice. BV (1 mg/kg ; 1, 2, and 3 times) was administered into the SC layer of the hind paw, and the antiallodynic effects were investigated using the von Frey test and by measuring the expression of neurokinin type 1 (NK-1) receptors in dorsal root ganglia (DRG). The administration of BV dose-dependently reduced the pain withdrawal threshold to mechanical stimuli compared with the pre-administration value and with that of the control group. After the development of the CPIP model, the expression of NK-1 receptors in DRG increased and then decreased following the administration of BV. SC administration of BV results in the attenuation of allodynia in a mouse model of CPIP. The antiallodynic effect was objectively proven through a reduction in the increased expression of NK-1 receptors in DRG.

  1. Investigating Reports of Complex Regional Pain Syndrome: An Analysis of HPV-16/18-Adjuvanted Vaccine Post-Licensure Data

    Science.gov (United States)

    Huygen, Frank; Verschueren, Kristin; McCabe, Candida; Stegmann, Jens-Ulrich; Zima, Julia; Mahaux, Olivia; Van Holle, Lionel; Angelo, Maria-Genalin

    2015-01-01

    Complex regional pain syndrome (CRPS) is a chronic pain disorder that typically follows trauma or surgery. Suspected CRPS reported after vaccination with human papillomavirus (HPV) vaccines led to temporary suspension of proactive recommendation of HPV vaccination in Japan. We investigated the potential CRPS signal in relation to HPV-16/18-adjuvanted vaccine (Cervarix®) by database review of CRPS cases with independent expert confirmation; a disproportionality analysis and analyses of temporality; an observed versus expected analysis using published background incidence rates; systematic reviews of aggregate safety data, and a literature review. The analysis included 17 case reports of CRPS: 10 from Japan (0.14/100,000 doses distributed) and seven from the United Kingdom (0.08/100,000). Five cases were considered by independent experts to be confirmed CRPS. Quantitative analyses did not suggest an association between CRPS and HPV-16/18-adjuvanted vaccine. Observed CRPS incidence after HPV-16/18 vaccination was statistically significantly below expected rates. Systematic database reviews using search terms varying in specificity and sensitivity did not identify new cases. No CRPS was reported during clinical development and no unexpected results found in the literature. There is not sufficient evidence to suggest an increased risk of developing CRPS following vaccination with HPV-16/18-adjuvanted vaccine. Post-licensure safety surveillance confirms the acceptable benefit-risk of HPV-16/18 vaccination. PMID:26501109

  2. [Comparable disorder of the body schema in patients with complex regional pain syndrome (CRPS) and phantom pain].

    Science.gov (United States)

    Reinersmann, A; Haarmeyer, G S; Blankenburg, M; Frettlöh, J; Krumova, E K; Ocklenburg, S; Maier, C

    2011-09-01

    In patients with complex regional pain syndrome (CRPS) a disruption of the body schema has been shown in an altered cortical representation of the hand and in delayed reaction times (RT) in the hand laterality recognition task. However, the role of attentional processes or the effect of isolated limb laterality training has not yet been clarified. The performance of healthy subjects (n = 38), CRPS patients (n = 12) and phantom limb pain (PLP) patients (n = 12) in a test battery of attentional performance (TAP) and in a limb laterality recognition task was compared and the effect of limb laterality training in CRPS patients and healthy subjects evaluated. The RTs of both CRPS and PLP patients were significantly slower than those of healthy subjects despite normal TAP values. The CRPS and PLP patients showed bilaterally delayed RTs. Through training RTs improved significantly but the RTs of CRPS patients remained slower than those of healthy subjects. In this study an equal disruption of the body schema was found in both CRPS and PLP patients which cannot be accounted for by attentional processes. For CRPS patients this disorder cannot be fully reversed by isolated limb laterality recognition training.

  3. Establishing the characteristics for patients with chronic Complex Regional Pain Syndrome: the value of the CRPS-UK Registry.

    Science.gov (United States)

    Shenker, Nicholas; Goebel, Andreas; Rockett, Mark; Batchelor, James; Jones, Gareth T; Parker, Richard; de C Williams, Amanda C; McCabe, Candida

    2015-05-01

    The long-term prognosis of patients with Complex Regional Pain Syndrome (CRPS) is unknown with no reported prospective studies from the United Kingdom longer than 18 months. The CRPS-UK Network aims to study this by use of a Registry. The aims of this article are, to outline the CRPS-UK Registry, assess the validity of the data and to describe the characteristics of a sample of the UK CRPS population. A web-based CRPS-UK Registry was developed and made accessible to centres experienced in diagnosing and managing patients with CRPS. Pragmatic annual follow-up questions were agreed. Up until July 2013, the Registry has recruited 240 patients. A blinded, validation study of 20 consecutive patients from two centres (10 each) demonstrated 95.6% completion and 99.4% accuracy of a random sample of the recorded data. These patients had chronic disease (median duration: 29 months); 72.5% were female (2.6:1), with a mean age at symptoms onset of 43 years, and were left-handed more than expected (21.8% versus 10% in the general population). Patients reported a delayed diagnosis, with the median time between symptom onset and diagnosis of 6 months. In all, 30 patients (12.5%) had multiple limb involvement and (83.3%) had a contiguous spread of CRPS. CRPS-UK Registry is a validated method for actively recruiting well-characterised patients with CRPS to provide further information on the long-term outcome.

  4. Novel Signs and Their Clinical Utility in Diagnosing Complex Regional Pain Syndrome (CRPS): A Prospective Observational Cohort Study.

    Science.gov (United States)

    Kuttikat, Anoop; Shaikh, Maliha; Oomatia, Amin; Parker, Richard; Shenker, Nicholas

    2017-06-01

    Delays in diagnosis occur with complex regional pain syndrome (CRPS). We define and prospectively demonstrate that novel bedside tests measuring body perception disruption can identify patients with CRPS postfracture. The objectives of our study were to define and validate 4 bedside tests, to identify the prevalence of positive tests in patients with CRPS and other chronic pain conditions, and to assess the clinical utility (sensitivity, specificity, positive predictive value, negative predictive value) for identifying CRPS within a Fracture cohort. This was a single UK teaching hospital prospective cohort study with 313 recruits from pain-free volunteers and patients with chronic pain conditions.Four novel tests were Finger Perception (FP), Hand Laterality identification (HL), Astereognosis (AS), and Body Scheme (BS) report. Five questionnaires (Brief Pain Inventory, Upper Extremity Functional Index, Lower Extremity Functional Index, Neglect-like Symptom Questionnaire, Hospital Anxiety and Depression Score) assessed the multidimensional pain experience. FP and BS were the best performing tests. Prospective monitoring of fracture patients showed that out of 7 fracture patients (total n=47) who had both finger misperception and abnormal BS report at initial testing, 3 developed persistent pain with 1 having a formal diagnosis of CRPS. Novel signs are reliable, easy to perform, and present in chronic pain patients. FP and BS have significant clinical utility in predicting persistent pain in a fracture group thereby allowing targeted early intervention.

  5. A CRPS-IgG-transfer-trauma model reproducing inflammatory and positive sensory signs associated with complex regional pain syndrome.

    Science.gov (United States)

    Tékus, Valéria; Hajna, Zsófia; Borbély, Éva; Markovics, Adrienn; Bagoly, Teréz; Szolcsányi, János; Thompson, Victoria; Kemény, Ágnes; Helyes, Zsuzsanna; Goebel, Andreas

    2014-02-01

    The aetiology of complex regional pain syndrome (CRPS), a highly painful, usually post-traumatic condition affecting the limbs, is unknown, but recent results have suggested an autoimmune contribution. To confirm a role for pathogenic autoantibodies, we established a passive-transfer trauma model. Prior to undergoing incision of hind limb plantar skin and muscle, mice were injected either with serum IgG obtained from chronic CRPS patients or matched healthy volunteers, or with saline. Unilateral hind limb plantar skin and muscle incision was performed to induce typical, mild tissue injury. Mechanical hyperalgesia, paw swelling, heat and cold sensitivity, weight-bearing ability, locomotor activity, motor coordination, paw temperature, and body weight were investigated for 8days. After sacrifice, proinflammatory sensory neuropeptides and cytokines were measured in paw tissues. CRPS patient IgG treatment significantly increased hind limb mechanical hyperalgesia and oedema in the incised paw compared with IgG from healthy subjects or saline. Plantar incision induced a remarkable elevation of substance P immunoreactivity on day 8, which was significantly increased by CRPS-IgG. In this IgG-transfer-trauma model for CRPS, serum IgG from chronic CRPS patients induced clinical and laboratory features resembling the human disease. These results support the hypothesis that autoantibodies may contribute to the pathophysiology of CRPS, and that autoantibody-removing therapies may be effective treatments for long-standing CRPS. Copyright © 2013 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  6. The place of occupational therapy in rehabilitation strategies of complex regional pain syndrome: Comparative study of 60 cases.

    Science.gov (United States)

    Rome, L

    2016-10-01

    The purpose of the study was to assess the value of combining occupational therapy (OT) with physical therapy (PT) for the rehabilitation of complex regional pain syndrome (CRPS) and to measure its effectiveness on activities of daily life. Sixty patients with CRPS type 1 were recruited and interviewed between September 1, 2014 and February 1, 2015. Thirty patients had undergone PT and thirty had undergone PT+OT. They were administered the short-form of the "Assessment of Life Habits" questionnaire (v.3.0 LIFE-H) created in Canada. This questionnaire consists of 16 items exploring activities of daily living, which were used to compare the effectiveness of the two rehabilitation protocols. The results of each test were submitted to the Wilcoxon test. After confirming the complexity of CRPS in terms of its etiology, clinical signs and progression, rehabilitation was effective, especially for pain. The patients who received PT+OT had on average 10% better dressing and undressing function, 25% better for meal preparation, and 20% better on personal care than those who underwent PT only. In CRPS, OT combined with PT brings a real benefit in restoring the essential activities of daily life. This strategy could be implemented as soon the diagnosis confirmed and continued for a very long time. It helps to avoid the risk of dependence on third parties. Copyright © 2016 SFCM. Published by Elsevier Masson SAS. All rights reserved.

  7. Knowledge and attitude towards the Middle East respiratory syndrome coronavirus among healthcare personnel in the southern region of Saudi Arabia.

    Science.gov (United States)

    Abbag, Huda F; El-Mekki, Awad Ahmed; Al Bshabshe, Ali Aobaid Ali; Mahfouz, Ahmed A; Al-Dosry, Ahasen A; Mirdad, Rasha T; AlKhttabi, Nora F; Abbag, Lubna F

    2018-03-07

    Middle East respiratory syndrome coronavirus (MERS-CoV) belongs to the family Coronaviridae, and is named for the crown-like spikes on its surface. The clinical presentation of MERS-CoV infection ranges from asymptomatic to very severe disease, and the classical presentation includes fever, cough chills, sore throat, myalgia, and arthralgia. A cross-sectional study of 339 healthcare personnel was conducted over an 8-month period in the Aseer region of Saudi Arabia using a structured survey that included demographic information and questions testing participant's knowledge. Approximately two-thirds of the respondents properly identified the causative agent of MERS-CoV as an RNA virus (66.4%, n=225) that is enveloped (68.1%, n=231). On the other hand, few respondents identified the proper number of strains or the genus (16.5% and 17.4%, respectively). More than half of the study sample identified the disease as zoonotic (57.2%, n=194). Similarly, 89.1% (n=302) identified that camels and bats are prone to infection with coronaviruses. Only 23.9% (n=81) properly identified March through May as the season with the highest transmission rate. There was a massive lack of adequate knowledge regarding prevalence of antibodies. Only 18.3% (n=62) of respondents identified PCR as the proper diagnostic confirmatory test for MERS-CoV infection. Regarding MERS-CoV clinical features, 76.4% (n=259) recognized the presence of sub-clinical infection, 64.7% (n=218) indicated that cases should be immediately isolated, and 46.9% (n=159) identified the main cause of mortality as respiratory failure. There is limited microbiological and virological knowledge of MERS-CoV infection among healthcare personnel in the southern region of Saudi Arabia, although the clinical aspects are known. Copyright © 2018. Published by Elsevier Ltd.

  8. Lung Microbiota Is Related to Smoking Status and to Development of Acute Respiratory Distress Syndrome in Critically Ill Trauma Patients.

    Science.gov (United States)

    Panzer, Ariane R; Lynch, Susan V; Langelier, Chaz; Christie, Jason D; McCauley, Kathryn; Nelson, Mary; Cheung, Christopher K; Benowitz, Neal L; Cohen, Mitchell J; Calfee, Carolyn S

    2018-03-01

    Cigarette smoking is associated with increased risk of acute respiratory distress syndrome (ARDS) in patients after severe trauma; however, the mechanisms underlying this association are unknown. To determine whether cigarette smoking contributes to ARDS development after trauma by altering community composition of the lung microbiota. We studied the lung microbiota of mechanically ventilated patients admitted to the ICU after severe blunt trauma. To do so, we used 16S ribosomal RNA gene amplicon sequencing of endotracheal aspirate samples obtained on ICU admission (n = 74) and at 48 hours after admission (n = 30). Cigarette smoke exposure (quantified using plasma cotinine), ARDS development, and other clinical parameters were correlated with lung microbiota composition. Smoking status was significantly associated with lung bacterial community composition at ICU admission (P = 0.007 by permutational multivariate ANOVA [PERMANOVA]) and at 48 hours (P = 0.03 by PERMANOVA), as well as with significant enrichment of potential pathogens, including Streptococcus, Fusobacterium, Prevotella, Haemophilus, and Treponema. ARDS development was associated with lung community composition at 48 hours (P = 0.04 by PERMANOVA) and was characterized by relative enrichment of Enterobacteriaceae and of specific taxa enriched at baseline in smokers, including Prevotella and Fusobacterium. After severe blunt trauma, a history of smoking is related to lung microbiota composition, both at the time of ICU admission and at 48 hours. ARDS development is also correlated with respiratory microbial community structure at 48 hours and with taxa that are relatively enriched in smokers at ICU admission. The data derived from this pilot study suggest that smoking-related changes in the lung microbiota could be related to ARDS development after severe trauma.

  9. Focus on metabolic and nutritional correlates of polycystic ovary syndrome and update on nutritional management of these critical phenomena.

    Science.gov (United States)

    Rondanelli, Mariangela; Perna, Simone; Faliva, Milena; Monteferrario, Francesca; Repaci, Erica; Allieri, Francesca

    2014-12-01

    Polycystic ovary syndrome (PCOS) is associated with numerous metabolic morbidities (insulin resistance (IR), central obesity) and various nutritional abnormalities (vitamin D deficit, mineral milieu alterations, omega6/omega3 PUFA ratio unbalance). We performed a systematic literature review to evaluate the till-now evidence regarding: (1) the metabolic and nutritional correlates of PCOS; (2) the optimum diet therapy for the treatment of these abnormalities. This review included 127 eligible studies. In addition to the well-recognized link between PCOS and IR, the recent literature underlines that in PCOS there is an unbalance in adipokines (adiponectin, leptin, visfatin) production and in omega6/omega3 PUFA ratio. Given the detrimental effect of overweight on these metabolic abnormalities, a change in the lifestyle must be the cornerstone in the treatment of PCOS patients. The optimum diet therapy for the PCOS treatment must aim at achieving specific metabolic goals, such as IR improvement, adipokines secretion and reproductive function. These goals must be reached through: accession of the patient to hypocaloric dietary program aimed at achieving and/or maintaining body weight; limiting the consumption of sugar and refined carbohydrates, preferring those with lower glycemic index; dividing the food intake in small and frequent meals, with high caloric intake at breakfast; increasing their intake of fish (4 times/week) or taking omega3 PUFA supplements; taking Vitamin D and chromium supplementation, if there are low serum levels. Lifestyle intervention remains the optimal treatment strategy for PCOS women. A relatively small weight loss (5 %) can improve IR, hyperandrogenism, menstrual function, fertility.

  10. Radiotracer transit measurements as an index of regional cerebral blood flow. Pt. 2. Results in acute alcohol withdrawal syndromes

    International Nuclear Information System (INIS)

    Dobrzanski, T.

    1975-01-01

    The data obtained in 72 male chronic alcoholics with acute alcohol withdrawal syndroms give evidence that there is a significant correlation between the numerical value of the cerebral radiorheographic index and the severity of the psychopathological syndrome (especially of the clouding of sensorium) in these patients. (author)

  11. Patients ≥ 75 years with acute coronary syndrome but without critical epicardial coronary disease: prevalence, characteristics, and outcome

    Science.gov (United States)

    Wong, Vincent; Farah, Ahmed; von Korn, Hubertus; Memisevic, Nedim; Richter, Stefan; Tukhiashvili, Ketevan; Lauer, Bernward; Ohlow, Marc-Alexander

    2015-01-01

    Objective Absence of significant epicardial coronary artery disease (CAD) in patients with acute onset of chest pain and elevation of myocardial necrosis markers is occasionally observed. The aim of this study was to analyse the clinical characteristics and outcome of such patients with advanced age. Methods We retrospectively analysed 4,311 patients with acute onset of chest pain plus necrosis marker elevation. Two hundred and seventy two patients without CAD on angiogram (6.3%) were identified. Out of them, 50 (1.2%) patients ≥ 75 years (Group I) were compared with (1) 222 acute coronary syndrome (ACS) patients without CAD on angiogram < 75 years (Group II), and (2) 610 consecutive patients ≥ 75 years with Non-ST-elevation Myocardial Infarction (NSTEMI) undergoing percutaneous coronary intervention (Group III). Results Group I compared to Group III patients made up for more females (64.0% vs. 49.2%; P < 0.0001), and had more severe anginal symptoms on presentation [Canadian Cardiovascular Society (CCS) class I/II, 26.0% vs. 49.8%; P = 0.02]. Group I patients also had lower troponin levels (0.62 ± 0.8 ng/mL vs. 27 ± 74 ng/mL; P < 0.02), lower leukocyte count (9.4 ± 3.13 × 109 vs. 12 ± 5.1 × 109; P = 0.001) and better preserved left ventricular function (56.7% ± 14.3% vs. 45% ± 11%; P < 0.0001). Event-free survival (cardiac death, myocardial infarction, recurrent angina, and re-hospitalisation) was more frequent in Group I and II patients compared to Group III patients (64.9%, 66.7%, and 41.6%, respectively; P < 0.0001). Conclusions ACS in patients ≥ 75 years without CAD is very infrequent, associated with a (1) similar outcome compared to ACS patients < 75 years without CAD, and (2) significant better outcome compared to NSTEMI patients ≥ 75 years. PMID:25678899

  12. Racial and ethnic differences in the prevalence of metabolic syndrome and its components of metabolic syndrome in women with polycystic ovary syndrome: a regional cross-sectional study.

    Science.gov (United States)

    Chan, Jessica L; Kar, Sujata; Vanky, Eszter; Morin-Papunen, Laure; Piltonen, Terhi; Puurunen, Johanna; Tapanainen, Juha S; Maciel, Gustavo Arantes Rosa; Hayashida, Sylvia Asaka Yamashita; Soares, Jose Maria; Baracat, Edmund Chada; Mellembakken, Jan Roar; Dokras, Anuja

    2017-08-01

    Polycystic ovary syndrome is a heterogeneous disorder and its presentation varies with race and ethnicity. Reproductive-age women with polycystic ovary syndrome are at increased risk of metabolic syndrome; however, it is not clear if prevalence of metabolic syndrome and clustering of its components differs based on race and ethnicity. Moreover, the majority of these women do not undergo routine screening for metabolic syndrome. We sought to compare the prevalence of metabolic syndrome and clustering of its components in women with polycystic ovary syndrome in the United States with women in India, Brazil, Finland, and Norway. This is a cross-sectional study performed in 1089 women with polycystic ovary syndrome from 1999 through 2016 in 5 outpatient clinics in the United States, India, Brazil, Finland, and Norway. Polycystic ovary syndrome was defined by the Rotterdam criteria. Main outcome measures were: metabolic syndrome prevalence, blood pressure, body mass index, fasting high-density lipoprotein cholesterol, fasting triglycerides, and fasting glucose. Data from all sites were reevaluated for appropriate application of diagnostic criteria for polycystic ovary syndrome, identification of polycystic ovary syndrome phenotype, and complete metabolic workup. The US White women with polycystic ovary syndrome were used as the referent group. Logistic regression models were used to evaluate associations between race and metabolic syndrome prevalence and its components and to adjust for potential confounders, including age and body mass index. The median age of the entire cohort was 28 years. Women from India had the highest mean Ferriman-Gallwey score for clinical hyperandrogenism (15.6 ± 6.5, P metabolic syndrome was highest in US Black women at 4.52 (95% confidence interval, 2.46-8.35) compared with US White women. When adjusted for age and body mass index, the prevalence was similar in the 2 groups. Significantly more Black women met body mass index and blood

  13. Reversible Stress Cardiomyopathy Presenting as Acute Coronary Syndrome with Elevated Troponin in the Absence of Regional Wall Motion Abnormalities: A Forme Fruste of Stress Cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Mahesh Anantha Narayanan

    2014-01-01

    Full Text Available We present a case of reversible stress cardiomyopathy in a surgical patient, described here as a forme fruste due to its atypical features. It is important to recognize such unusual presentation of stress cardiomyopathy that mimics acute coronary syndrome. Stress cardiomyopathy commonly presents as acute coronary syndrome and is characterized by typical or atypical variants of regional wall motion abnormalities. We report a 60-year-old Caucasian male with reversible stress cardiomyopathy following a sternal fracture fixation. Although the patient had several typical features of stress cardiomyopathy including physical stress, ST-segment elevation, elevated cardiac biomarkers and normal epicardial coronaries, there were few features that were atypical, including unusual age, gender, absence of regional wall motion abnormalities, high lateral ST elevation, and high troponin-ejection fraction product. In conclusion, this could represent a forme fruste of stress cardiomyopathy.

  14. Physical mapping of the Bloom syndrome region by the identification of YAC and P1 clones from human chromosome 15 band q26.1

    Energy Technology Data Exchange (ETDEWEB)

    Straughen, J.; Groden, J. [Univ. of Cincinnati College of Medicine, OH (United States); Ciocci, S. [New York Blood Center, NY (United States)] [and others

    1996-07-01

    The gene for Bloom syndrome (BLM) has been mapped to human chromosome 15 band q26.1 by homozygosity mapping. Further refinement of the location of BLM has relied upon linkage-disequilibrium mapping and somatic intragenic recombination. In combination with these mapping approaches and to identify novel DNA markers and probes for the BLM candidate region, a contiguous representation of the 2-Mb region that contains the BLM gene was generated and is presented here. YAC and P1 clones from the region have been identified and ordered by using previously available genetic markers in the region along with newly developed sequence-tagged sites from radiation-restriction map of the 2-Mb region that allowed estimation of the distance between polymorphic microsatellite loci is also reported. This map and the DNA markers derived from it were instrumental in the recent identification of the BLM gene. 25 refs., 3 figs., 3 tabs.

  15. Bat white-nose syndrome: A real-time TaqMan polymerase chain reaction test targeting the intergenic spacer region of Geomyces destructans

    Science.gov (United States)

    Laura K Muller; Jeffrey M. Lorch; Daniel L. Lindner; Michael O' Connor; Andrea Gargas; David S. Blehert

    2013-01-01

    The fungus Geomyces destructans is the causative agent of white-nose syndrome (WNS), a disease that has killed millions of North American hibernating bats. We describe a real-time TaqMan PCR test that detects DNA from G. destructans by targeting a portion of the multicopy intergenic spacer region of the rRNA gene complex. The...

  16. Are Pain-Related Fears Mediators for Reducing Disability and Pain in Patients with Complex Regional Pain Syndrome Type 1? An Explorative Analysis on Pain Exposure Physical Therapy

    Science.gov (United States)

    Barnhoorn, Karlijn J.; Staal, J. Bart; van Dongen, Robert T. M.; Frölke, Jan Paul M.; Klomp, Frank P.; van de Meent, Henk; Samwel, Han; Nijhuis-van der Sanden, Maria W. G.

    2015-01-01

    Objective To investigate whether pain-related fears are mediators for reducing disability and pain in patients with Complex Regional Pain Syndrome type 1 when treating with Pain Exposure Physical Therapy. Design An explorative secondary analysis of a randomised controlled trial. Participants Fifty-six patients with Complex Regional Pain Syndrome type 1. Interventions The experimental group received Pain Exposure Physical Therapy in a maximum of five treatment sessions; the control group received conventional treatment following the Dutch multidisciplinary guideline. Outcome measures Levels of disability, pain, and pain-related fears (fear-avoidance beliefs, pain catastrophizing, and kinesiophobia) were measured at baseline and after 3, 6, and 9 months follow-up. Results The experimental group had a significantly larger decrease in disability of 7.77 points (95% CI 1.09 to 14.45) and in pain of 1.83 points (95% CI 0.44 to 3.23) over nine months than the control group. The potential mediators pain-related fears decreased significantly in both groups, but there were no significant differences between groups, which indicated that there was no mediation. Conclusion The reduction of pain-related fears was comparable in both groups. We found no indication that pain-related fears mediate the larger reduction of disability and pain in patients with Complex Regional Pain Syndrome type 1 treated with Pain Exposure Physical Therapy compared to conventional treatment. Trial registration International Clinical Trials Registry NCT00817128 PMID:25919011

  17. Interstitial deletion in the "critical region" of the long arm of the X chromosome in a mentally retarded boy and his normal mother

    DEFF Research Database (Denmark)

    Tabor, A; Andersen, O; Lundsteen, C

    1983-01-01

    A family in which an intestitial deletion of the X chromosome, del(X)(q13q21.3), is segregating was ascertained through a boy with cleft lip and palate, agenesis of the corpus callosum, and severe mental retardation. The possible causal relationship to his chromosome abnormality is discussed. Alt....... Although the deletion occurred within the critical region, the mother showed no signs of gonadal dysgenesis. A phenotypically normal daughter was, as her mother, monosomic for this region of the X, and both showed random inactivation of the X chromosome....

  18. An Individual with Gilles de la Tourette Syndrome and Smith-Magenis Microdeletion Syndrome: Is Chromosome 17p11.2 a Candidate Region for Tourette Syndrome Putative Susceptibility Genes?

    Science.gov (United States)

    Shelley, B. P.; Robertson, M. M.; Turk, J.

    2007-01-01

    This is the first published case description in the current literature of the association of definite Gilles de la Tourette syndrome (GTS) and the Smith-Magenis syndrome (SMS), both confirmed by DSM-IV-TR criteria and molecular cytogenetic analysis, respectively. The co-occurrence of GTS, SMS and their common behavioural/neuropsychiatric…

  19. Testing the validity of preventing chronic regional pain syndrome with vitamin C after distal radius fracture. [Corrected].

    Science.gov (United States)

    Malay, Sunitha; Chung, Kevin C

    2014-11-01

    The American Academy of Orthopaedic Surgeons recommends the use of vitamin C to prevent complex regional pain syndrome (CRPS) for patients with distal radius fractures (DRFs). We hypothesized that the evidence for supporting this recommendation is weak, based on epidemiological principles of association and causality. The specific aim of this project was to test the validity of this recommendation. We conducted a literature review to retrieve articles reporting on the use of vitamin C to prevent CRPS. Data collected included sample size, study design type, dose of vitamin C used, and outcome measures of association expressed as relative risk (RR) and odds ratio. We then applied Hill criteria to evaluate the relationship between vitamin C and CRPS. We obtained 225 articles from the database search. After the exclusion of duplicates, unrelated articles, editorial letters, and commentaries, we found 4 articles and 1 systematic review relevant to our topic. Six of the 9 Hill criteria were met, and an earlier meta-analysis showed a quantified reduction in CRPS risk. However, criteria like biological plausibility, specificity, and coherence were not met. The number of causal/association criteria met was adequate to support the scientific premise of the effect of vitamin C in preventing CRPS after DRF. Furthermore, vitamin C administration is of relatively low cost and has few complications unless administered in large doses. Owing to sufficient epidemiological evidence availability, the American Academy of Orthopaedic Surgeons recommendation of vitamin C to prevent CRPS has practical merit. Therapeutic II. Copyright © 2014 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

  20. Prevalence of Metabolic Syndrome in Adult Population of District of Tetovo in the Northwest Region of the Republic of Macedonia

    Directory of Open Access Journals (Sweden)

    Atila Rexhepi

    2018-03-01

    Full Text Available Objective: To estimate the prevalence of metabolic syndrome (MetS and its separate components in the adult population of the district of Tetovo. Methods: Total of 630 subjects aged over 18 years were selected at random, from the primary healthcare register, to constitute a representative sample of a population in the district of Tetovo, adjusted for age and sex. MetS is defined according to the definition of the National Cholesterol Education Program Adult Treatment Panel III (NCEP ATPIII. Results: The prevalence of MetS in the district of Tetovo was 28.25%. It was significantly higher in women than in men (34.06% versus 22.26%, p=0.004. The largest number of people with MetS living in rural areas (31.32% versus 23.60%, p=0.002. Of the particular components of MetS, 52.06% of the population had increased arterial blood pressure, 46.35% reduced values of high-density lipoprotein cholesterol (HDL-C and 43.17% abdominal adiposity (AA. In women population, more dominated was abdominal adiposity (59.38% and 26.54%, p=0.000, in men more dominated was increased arterial blood pressure (53.44% and 50.65%, p=0.48. Conclusion: The prevalence of MetS in our population is high, particularly in women. The prevalence rates for arterial hypertension and abdominal obesity are among the highest reported for the region. Advice on diet and change in lifestyles, in addition to the pharmacological treatment of this category, would contribute to the prevention of cardiovascular disease, type II diabetes and complications from these diseases.

  1. Relationship Between HbA1c and Complex Regional Pain Syndrome in Stroke Patients With Type 2 Diabetes Mellitus

    Science.gov (United States)

    2016-01-01

    Objective To investigate the relationship between glycosylated hemoglobin A (HbA1c) and complex regional pain syndrome (CRPS) in stroke patients with type 2 diabetes mellitus (T2DM). Methods A retrospective chart review was performed of stroke patients from January 2012 to December 2013. We reviewed 331 patients and included 200 in the analysis. We divided them into CRPS and non-CRPS groups and compared them by age, gender, stroke lesion, cause of stroke, duration of T2DM, HbA1c (%), National Institutes of Health Stroke Scale score, affected shoulder flexor muscle strength, Fugl-Meyer Assessment score, motricity index, Functional Independence Measure, Korean version of Modified Barthel Index, blood glucose level on admission day, duration from stroke onset to HbA1c check, and duration from stroke onset to three-phase bone scan for CRPS diagnosis. Thereafter, we classified the patients into five groups by HbA1c level (group 1, 5.0%–5.9%; group 2, 6.0%–6.9%; group 3, 7.0%–7.9%; group 4, 8.0%–8.9%; and group 5, 9.0%–9.9%) and we investigated the difference in CRPS prevalence between the two groups. Results Of the 200 patients, 108 were in the CRPS group and 92 were in the non-CRPS group. There were significant differences in HbA1c (pCRPS prevalence (pCRPS prevalence and thus that uncontrolled blood glucose can affect CRPS occurrence in stroke patients with diabetes. PMID:27847707

  2. Anti tumor necrosis factor - alpha adalimumab for complex regional pain syndrome type 1 (CRPS-I): a case series.

    Science.gov (United States)

    Eisenberg, Elon; Sandler, Ifat; Treister, Roi; Suzan, Erica; Haddad, May

    2013-11-01

    Evidence suggests tumor necrosis factor-alpha (TNF-α) mediates, at least in part, symptoms and signs in complex regional pain syndrome (CRPS). Here, we present a case series of patients with CRPS type 1, in whom the response to the anti-TNF-α adalimumab was assessed. Ten patients with CRPS type 1 were recruited. Assessments were performed before treatment, at 1 week, and 1, 3, and 6 months following 3 biweekly subcutaneous injections (40 mg/0.8 mL) adalimumab (Humira(®) ) and included the followings: Pain intensity using a 0-10 cm visual analog scale; the Short Form of the McGill Pain Questionnaire; the Beck Depression Inventory; the SF-36 questionnaire and mechanical and thermal thresholds (Von frey hair and Thermal Sensory Analyzer, respectively). In addition to the description of individual patient responses, both intention to treat (ITT) and per-protocol (PP) analyses were performed for the entire group. Three subgroups of patients were identified (3 patients in each): "nonresponders", "partial responders", and "robust responders" in whom improvement in almost all parameters was noted. Both the ITT and PP analyses demonstrated only a trend toward improvement in mechanical pain thresholds following treatment (ITT χ² = 13.83, P = 0.008; PP χ² = 10.29, P = 0.036). These results suggest adalimumab, and possibly other anti-TNF-α, can be potentially useful in some (although not in all) patients with CRPS type 1. These preliminary results along with the growing body of evidence which points to the involvement of TNF-α in the pathogenesis of CRPS justify further studies in this area. © 2013 World Institute of Pain.

  3. Diagnostic Performance of Three Phase Bone Scan for Complex Regional Pain Syndrome Type 1 with Optimally Modified Image Criteria

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Hyun Woo; Paeng, Jin Chul; Nahm, Francins Sahngun; Kim, Seog Gyun; Zehra, Tanzeel; Oh, So Won; Lee, Hyo Sang; Kang, Keon Wook; Chung, June Key; Lee, Myung Chul; Lee, Dong Soo [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    2011-12-15

    Although the three phase bone scan (TBPS) is one of the widely used imaging studies for diagnosing complex regional pain syndrome type 1 (CRPS 1), there is some controversy regarding the TPBS image criteria for CRPS 1. In this study, we modified the image criteria using image pattern and quantitative analysis in the patients diagnosed using the most recent consensus clinical diagnostic criteria. The study included 140 patients with suspected CRPS 1 (CRPS 1, n=79; non CRPS, n=61; mean age 39{+-}15 years) who underwent TPBS. The clinical diagnostic criteria for CRPS 1 revised by the Budapest consensus group were used for confirmative diagnosis. Patients were classified according to flow/pool and delayed uptake (DU) image patterns, and the time interval between the initiating event and TPBS (TI{sup eventscan)}. Quantitative analysis for lesion to contralateral ratio (LCR) was performed. Modified TPBS image criteria were created and evaluated for optimal diagnostic performance. Both increased and decreased periarticular DU were significant image findings for CRPS 1 (CRPS 1 positive rate=73% in the increased DU group, 75% in the decreased DU group). The TI{sup eventscand}id not differ significantly between the different image pattern groups. Quantitative analysis revealed an LCR of 1.43 was the optimal cutoff value for CRPS 1 and diagnostic performance was significantly improved in the increased DU group (area under the curve=0.732). Given the modified image criteria, the sensitivity and specificity of TPBS for diagnosing CRPS 1 were 80% and 72%, respectively. Optimally modified TPBS image criteria for CRPS 1 were suggested using image pattern and quantitative analysis. With the criteria, TPBS is an effective imaging study for CRPS 1 even with the most recent consensus clinical diagnostic criteria.

  4. Bodily Sensory Inputs and Anomalous Bodily Experiences in Complex Regional Pain Syndrome: Evaluation of the Potential Effects of Sound Feedback

    Directory of Open Access Journals (Sweden)

    Ana Tajadura-Jiménez

    2017-07-01

    Full Text Available Neuroscientific studies have shown that human's mental body representations are not fixed but are constantly updated through sensory feedback, including sound feedback. This suggests potential new therapeutic sensory approaches for patients experiencing body-perception disturbances (BPD. BPD can occur in association with chronic pain, for example in Complex Regional Pain Syndrome (CRPS. BPD often impacts on emotional, social, and motor functioning. Here we present the results from a proof-of-principle pilot study investigating the potential value of using sound feedback for altering BPD and its related emotional state and motor behavior in those with CRPS. We build on previous findings that real-time alteration of the sounds produced by walking can alter healthy people's perception of their own body size, while also resulting in more active gait patterns and a more positive emotional state. In the present study we quantified the emotional state, BPD, pain levels and gait of twelve people with CRPS Type 1, who were exposed to real-time alteration of their walking sounds. Results confirm previous reports of the complexity of the BPD linked to CRPS, as participants could be classified into four BPD subgroups according to how they mentally visualize their body. Further, results suggest that sound feedback may affect the perceived size of the CRPS affected limb and the pain experienced, but that the effects may differ according to the type of BPD. Sound feedback affected CRPS descriptors and other bodily feelings and emotions including feelings of emotional dominance, limb detachment, position awareness, attention and negative feelings toward the limb. Gait also varied with sound feedback, affecting the foot contact time with the ground in a way consistent with experienced changes in body weight. Although, findings from this small pilot study should be interpreted with caution, they suggest potential applications for regenerating BDP and its related

  5. Complete recovery from intractable complex regional pain syndrome, CRPS-type I, following anesthetic ketamine and midazolam.

    Science.gov (United States)

    Kiefer, Ralph-Thomas; Rohr, Peter; Ploppa, Annette; Altemeyer, Karl-Heinz; Schwartzman, Robert Jay

    2007-06-01

    To describe the treatment of an intractable complex regional pain syndrome I (CRPS-I) patient with anesthetic doses of ketamine supplemented with midazolam. A patient presented with a rapidly progressing contiguous spread of CRPS from a severe ligamentous wrist injury. Standard pharmacological and interventional therapy successively failed to halt the spread of CRPS from the wrist to the entire right arm. Her pain was unmanageable with all standard therapy. As a last treatment option, the patient was transferred to the intensive care unit and treated on a compassionate care basis with anesthetic doses of ketamine in gradually increasing (3-5 mg/kg/h) doses in conjunction with midazolam over a period of 5 days. On the second day of the ketamine and midazolam infusion, edema, and discoloration began to resolve and increased spontaneous movement was noted. On day 6, symptoms completely resolved and infusions were tapered. The patient emerged from anesthesia completely free of pain and associated CRPS signs and symptoms. The patient has maintained this complete remission from CRPS for 8 years now. In a patient with severe spreading and refractory CRPS, a complete and long-term remission from CRPS has been obtained utilizing ketamine and midazolam in anesthetic doses. This intensive care procedure has very serious risks but no severe complications occurred. The psychiatric side effects of ketamine were successfully managed with the concomitant use of midazolam and resolved within 1 month of treatment. This case report illustrates the effectiveness and safety of high-dose ketamine in a patient with generalized, refractory CRPS.

  6. Recommendations for a first Core Outcome Measurement set for complex regional PAin syndrome Clinical sTudies (COMPACT)

    Science.gov (United States)

    Grieve, Sharon; Perez, Roberto SGM; Birklein, Frank; Brunner, Florian; Bruehl, Stephen; Harden R, Norman; Packham, Tara; Gobeil, Francois; Haigh, Richard; Holly, Janet; Terkelsen, Astrid; Davies, Lindsay; Lewis, Jennifer; Thomassen, Ilona; Connett, Robyn; Worth, Tina; Vatine, Jean-Jacques; McCabe, Candida S

    2017-01-01

    Complex Regional Pain Syndrome (CRPS) is a persistent pain condition that remains incompletely understood and challenging to treat. Historically, a wide range of different outcome measures have been used to capture the multidimensional nature of CRPS. This has been a significant limiting factor in the advancement of our understanding of the mechanisms and management of CRPS. In 2013, an international consortium of patients, clinicians, researchers and industry representatives was established, to develop and agree on a minimum core set of standardised outcome measures for use in future CRPS clinical research, including but not limited to clinical trials within adult populations The development of a core measurement set was informed through workshops and supplementary work, using an iterative consensus process. ‘What is the clinical presentation and course of CRPS, and what factors influence it?’ was agreed as the most pertinent research question that our standardised set of patient-reported outcome measures should be selected to answer. The domains encompassing the key concepts necessary to answer the research question were agreed as: pain, disease severity, participation and physical function, emotional and psychological function, self efficacy, catastrophizing and patient's global impression of change. The final core measurement set included the optimum generic or condition-specific patient-reported questionnaire outcome measures, which captured the essence of each domain, and one clinician reported outcome measure to capture the degree of severity of CRPS. The next step is to test the feasibility and acceptability of collecting outcome measure data using the core measurement set in the CRPS population internationally. PMID:28178071

  7. The association between ACE inhibitors and the complex regional pain syndrome: Suggestions for a neuro-inflammatory pathogenesis of CRPS.

    Science.gov (United States)

    de Mos, M; Huygen, F J P M; Stricker, B H Ch; Dieleman, J P; Sturkenboom, M C J M

    2009-04-01

    Antihypertensive drugs interact with mediators that are also involved in complex regional pain syndrome (CRPS), such a neuropeptides, adrenergic receptors, and vascular tone modulators. Therefore, we aimed to study the association between the use of antihypertensive drugs and CRPS onset. We conducted a population-based case-control study in the Integrated Primary Care Information (IPCI) database in the Netherlands. Cases were identified from electronic records (1996-2005) and included if they were confirmed during an expert visit (using IASP criteria), or if they had been diagnosed by a medical specialist. Up to four controls per cases were selected, matched on gender, age, calendar time, and injury. Exposure to angiotensin converting enzyme (ACE) inhibitors, angiotensin II receptor antagonists, beta-blockers, calcium channel blockers, and diuretics was assessed from the automated prescription records. Data were analyzed using multivariate conditional logistic regression. A total of 186 cases were matched to 697 controls (102 confirmed during an expert visit plus 84 with a specialist diagnosis). Current use of ACE inhibitors was associated with an increased risk of CRPS (OR(adjusted): 2.7, 95% CI: 1.1-6.8). The association was stronger if ACE inhibitors were used for a longer time period (OR(adjusted): 3.0, 95% CI: 1.1-8.1) and in higher dosages (OR(adjusted): 4.3, 95% CI: 1.4-13.7). None of the other antihypertensive drug classes was significantly associated with CRPS. We conclude that ACE inhibitor use is associated with CRPS onset and hypothesize that ACE inhibitors influence the neuro-inflammatory mechanisms that underlie CRPS by their interaction with the catabolism of substance P and bradykinin.

  8. Bodily Sensory Inputs and Anomalous Bodily Experiences in Complex Regional Pain Syndrome: Evaluation of the Potential Effects of Sound Feedback

    Science.gov (United States)

    Tajadura-Jiménez, Ana; Cohen, Helen; Bianchi-Berthouze, Nadia

    2017-01-01

    Neuroscientific studies have shown that human's mental body representations are not fixed but are constantly updated through sensory feedback, including sound feedback. This suggests potential new therapeutic sensory approaches for patients experiencing body-perception disturbances (BPD). BPD can occur in association with chronic pain, for example in Complex Regional Pain Syndrome (CRPS). BPD often impacts on emotional, social, and motor functioning. Here we present the results from a proof-of-principle pilot study investigating the potential value of using sound feedback for altering BPD and its related emotional state and motor behavior in those with CRPS. We build on previous findings that real-time alteration of the sounds produced by walking can alter healthy people's perception of their own body size, while also resulting in more active gait patterns and a more positive emotional state. In the present study we quantified the emotional state, BPD, pain levels and gait of twelve people with CRPS Type 1, who were exposed to real-time alteration of their walking sounds. Results confirm previous reports of the complexity of the BPD linked to CRPS, as participants could be classified into four BPD subgroups according to how they mentally visualize their body. Further, results suggest that sound feedback may affect the perceived size of the CRPS affected limb and the pain experienced, but that the effects may differ according to the type of BPD. Sound feedback affected CRPS descriptors and other bodily feelings and emotions including feelings of emotional dominance, limb detachment, position awareness, attention and negative feelings toward the limb. Gait also varied with sound feedback, affecting the foot contact time with the ground in a way consistent with experienced changes in body weight. Although, findings from this small pilot study should be interpreted with caution, they suggest potential applications for regenerating BDP and its related bodily feelings in

  9. Role of NFkappaB in an animal model of complex regional pain syndrome-type I (CRPS-I).

    Science.gov (United States)

    de Mos, Marissa; Laferrière, André; Millecamps, Magali; Pilkington, Mercedes; Sturkenboom, Miriam C J M; Huygen, Frank J P M; Coderre, Terence J

    2009-11-01

    NFkappaB is involved in several pathogenic mechanisms that are believed to underlie the complex regional pain syndrome (CRPS), including ischemia, inflammation and sensitization. Chronic postischemia pain (CPIP) has been developed as an animal model that mimics the symptoms of CRPS-I. The possible involvement of NFkappaB in CRPS-I was studied using CPIP rats. Under sodium pentobarbital anesthesia, a tourniquet was placed around the rat left ankle joint, producing 3 hours of ischemia, followed by rapid reperfusion (IR injury). NFkappaB was measured in nuclear extracts of muscle and spinal cord tissue using ELISA. Moreover, the anti-allodynic (mechanical and cold) effect was tested for systemic, intrathecal, or intraplantar treatment with the NFkappaB inhibitor pyrrolidine dithiocarbamate (PDTC). At 2 and 48 hours after IR injury, NFkappaB was elevated in muscle and spinal cord of CPIP rats compared to shams. At 7 days, NFkappaB levels were normalized in muscle, but still elevated in spinal cord tissue. Systemic PDTC treatment relieved mechanical and cold allodynia in a dose-dependent manner, lasting for at least 3 hours. Intrathecal-but not intraplantar-administration also relieved mechanical allodynia. The results suggest that muscle and spinal NFkappaB plays a role in the pathogenesis of CPIP and potentially of human CRPS. Using the CPIP model, we demonstrate that NFkappaB is involved in the development of allodynia after a physical injury (ischemia and reperfusion) without direct nerve trauma. Since CPIP animals exhibit many features of human CRPS-I, this observation indicates a potential role for NFkappaB in human CRPS.

  10. Diagnostic Performance of Three Phase Bone Scan for Complex Regional Pain Syndrome Type 1 with Optimally Modified Image Criteria

    International Nuclear Information System (INIS)

    Kwon, Hyun Woo; Paeng, Jin Chul; Nahm, Francins Sahngun; Kim, Seog Gyun; Zehra, Tanzeel; Oh, So Won; Lee, Hyo Sang; Kang, Keon Wook; Chung, June Key; Lee, Myung Chul; Lee, Dong Soo

    2011-01-01

    Although the three phase bone scan (TBPS) is one of the widely used imaging studies for diagnosing complex regional pain syndrome type 1 (CRPS 1), there is some controversy regarding the TPBS image criteria for CRPS 1. In this study, we modified the image criteria using image pattern and quantitative analysis in the patients diagnosed using the most recent consensus clinical diagnostic criteria. The study included 140 patients with suspected CRPS 1 (CRPS 1, n=79; non CRPS, n=61; mean age 39±15 years) who underwent TPBS. The clinical diagnostic criteria for CRPS 1 revised by the Budapest consensus group were used for confirmative diagnosis. Patients were classified according to flow/pool and delayed uptake (DU) image patterns, and the time interval between the initiating event and TPBS (TI eventscan) . Quantitative analysis for lesion to contralateral ratio (LCR) was performed. Modified TPBS image criteria were created and evaluated for optimal diagnostic performance. Both increased and decreased periarticular DU were significant image findings for CRPS 1 (CRPS 1 positive rate=73% in the increased DU group, 75% in the decreased DU group). The TI eventscand id not differ significantly between the different image pattern groups. Quantitative analysis revealed an LCR of 1.43 was the optimal cutoff value for CRPS 1 and diagnostic performance was significantly improved in the increased DU group (area under the curve=0.732). Given the modified image criteria, the sensitivity and specificity of TPBS for diagnosing CRPS 1 were 80% and 72%, respectively. Optimally modified TPBS image criteria for CRPS 1 were suggested using image pattern and quantitative analysis. With the criteria, TPBS is an effective imaging study for CRPS 1 even with the most recent consensus clinical diagnostic criteria.

  11. High-Frequency Repetitive Sensory Stimulation as Intervention to Improve Sensory Loss in Patients with Complex Regional Pain Syndrome I.

    Science.gov (United States)

    David, Marianne; Dinse, Hubert R; Mainka, Tina; Tegenthoff, Martin; Maier, Christoph

    2015-01-01

    Achieving perceptual gains in healthy individuals or facilitating rehabilitation in patients is generally considered to require intense training to engage neuronal plasticity mechanisms. Recent work, however, suggested that beneficial outcome similar to training can be effectively acquired by a complementary approach in which the learning occurs in response to mere exposure to repetitive sensory stimulation (rSS). For example, high-frequency repetitive sensory stimulation (HF-rSS) enhances tactile performance and induces cortical reorganization in healthy subjects and patients after stroke. Patients with complex regional pain syndrome (CRPS) show impaired tactile performance associated with shrinkage of cortical maps. We here investigated the feasibility and efficacy of HF-rSS, and low-frequency rSS (LF-rSS) to enhance tactile performance and reduce pain intensity in 20 patients with CRPS type I. Intermittent high- or low-frequency electrical stimuli were applied for 45 min/day to all fingertips of the affected hand for 5 days. Main outcome measures were spatial two-point-discrimination thresholds and mechanical detection thresholds measured on the tip of the index finger bilaterally. Secondary endpoint was current pain intensity. All measures were assessed before and on day 5 after the last stimulation session. HF-rSS applied in 16 patients improved tactile discrimination on the affected hand significantly without changes contralaterally. Current pain intensity remained unchanged on average, but decreased in four patients by ≥30%. This limited pain relief might be due to the short stimulation period of 5 days only. In contrast, after LF-rSS, tactile discrimination was impaired in all four patients, while detection thresholds and pain were not affected. Our data suggest that HF-rSS could be used as a novel approach in CRPS treatment to improve sensory loss. Longer treatment periods might be required to induce consistent pain relief.

  12. The rubber hand illusion in complex regional pain syndrome: preserved ability to integrate a rubber hand indicates intact multisensory integration.

    Science.gov (United States)

    Reinersmann, Annika; Landwehrt, Julia; Krumova, Elena K; Peterburs, Jutta; Ocklenburg, Sebastian; Güntürkün, Onur; Maier, Christoph

    2013-09-01

    In patients with complex regional pain syndrome (CRPS) type 1, processing of static tactile stimuli is impaired, whereas more complex sensory integration functions appear preserved. This study investigated higher order multisensory integration of body-relevant stimuli using the rubber hand illusion in CRPS patients. Subjective self-reports and skin conductance responses to watching the rubber hand being harmed were compared among CRPS patients (N=24), patients with upper limb pain of other origin (N=21, clinical control group), and healthy subjects (N=24). Additionally, the influence of body representation (body plasticity [Trinity Assessment of Body Plasticity], neglect-like severity symptoms), and clinical signs of illusion strength were investigated. For statistical analysis, 1-way analysis of variance, t test, Pearson correlation, with α=0.05 were used. CRPS patients did not differ from healthy subjects and the control group with regard to their illusion strength as assessed by subjective reports or skin conductance response values. Stronger left-sided rubber hand illusions were reported by healthy subjects and left-side-affected CRPS patients. Moreover, for this subgroup, illness duration and illusion strength were negatively correlated. Overall, severity of neglect-like symptoms and clinical signs were not related to illusion strength. However, patients with CRPS of the right hand reported significantly stronger neglect-like symptoms and significantly lower illusion strength of the affected hand than patients with CRPS of the left hand. The weaker illusion of CRPS patients with strong neglect-like symptoms on the affected hand supports the role of top-down processes modulating body ownership. Moreover, the intact ability to perceive illusory ownership confirms the notion that, despite impaired processing of proprioceptive or tactile input, higher order multisensory integration is unaffected in CRPS. Copyright © 2013 International Association for the Study

  13. High frequency repetitive sensory stimulation as intervention to improve sensory loss in patients with complex regional pain syndrome (CRPS I

    Directory of Open Access Journals (Sweden)

    Marianne eDavid

    2015-11-01

    Full Text Available Achieving perceptual gains in healthy individuals, or facilitating rehabilitation in patients is generally considered to require intense training to engage neuronal plasticity mechanisms. Recent work, however, suggested that beneficial outcome similar to training can be effectively acquired by a complementary approach in which the learning occurs in response to mere exposure to repetitive sensory stimulation (rSS. For example, high-frequency repetitive sensory stimulation (HF-rSS enhances tactile performance and induces cortical reorganization in healthy subjects and patients after stroke. Patients with complex regional pain syndrome (CRPS show impaired tactile performance associated with shrinkage of cortical maps. We here investigated the feasibility and efficacy of HF-rSS, and low-frequency rSS (LF-rSS to enhance tactile performance and reduce pain intensity in 20 patients with CRPS type I. Intermittent high or low frequency electrical stimuli were applied for 45min/day to all fingertips of the affected hand for 5 days. Main outcome measures were spatial 2-point-discrimination thresholds and mechanical detection thresholds measured on the tip of the index finger bilaterally. Secondary endpoint was current pain intensity. All measures were assessed before and on day 5 after the last stimulation session. HF-rSS applied in 16 patients improved tactile discrimination on the affected hand significantly without changes contralaterally. Current pain intensity remained unchanged on average, but decreased in 4 patients by 30%. This limited pain relief might be due to the short stimulation period of 5 days only. In contrast, after LF-rSS, tactile discrimination was impaired in all 4 patients, while detection thresholds and pain were not affected. Our data suggest that HF-rSS could be used as a novel approach in CRPS treatment to improve sensory loss. Longer treatment periods might be required to induce consistent pain relief.

  14. Countries, Within-Country Regions, and Multiple-Country Regions in International Management: A Functional, Institutional, and Critical Event (FICE) Perspective

    DEFF Research Database (Denmark)

    Søndergaard, Mikael; Peterson, Mark F.

    2014-01-01

    We introduce the focused issue by offering a functional, institutional and critical event or FICE perspective on the relationship between cultural boundaries and the boundaries of modern nation states (termed countries here). Our perspective draws from three kinds of theory that suggest how...... governmental boundaries have come to be connected in different degrees and in different ways to cultural groups. We use the FICE perspective to integrate the four articles in the focused issue that speak to whether boundaries around countries matter as compared to boundaries around religious groups, within...

  15. Brief note and evaluation of acute-radiation syndrome and treatment of a Tokai-mura criticality accident patient

    Energy Technology Data Exchange (ETDEWEB)

    Ishii, Takeshi; Futami, Satoru; Nishida, Masamichi; Suzuki, Toru; Sakamoto, Tetsuya; Suzuki, Norio; Maekawa, Kazuhiko [Tokyo Univ. (Japan). Graduate School of Medicine

    2001-09-01

    Patient A who was exposed to a critical dose of radiation developed skin lesions throughout the body surface, gastrointestinal disorder with massive diarrhea and prominent bleeding, which caused severe loss in body fluids. Gastrointestinal bleeding due to the deteriorated intestinal mucosa was considered to be one of the major causes of death, although infection did not develop, possibly because of SDD and aseptic intensive care, until terminal stages. Patient A ultimately developed respiratory and renal failure in addition to skin exudate and gastrointestinal bleeding, and died of multiple organ failure on the 83rd day after exposure. The extreme unevenness of the dose distribution and the neutron versus {gamma}-ray component made the clinical manifestation very complicated. Initially, the mean absorbed dose was calculated as 16-20 GyEq for Patient A, mainly based on neutron-activated {sup 24}Na in the blood. However, a very recent calculation showed that the absorbed skin dose was highest at the upper-right abdomen reaching 61.8 Gy (27.0 as neutron plus 34.8 Gy as {gamma}-ray). The dorsal side was calculated to have received one eighth of the value of the abdominal side, and much smaller neutron component. His absorbed-dose distribution throughout the body was very inhomogeneous because of the closeness of the standing point to the mixing tank. Despite prolonged survival because of intensive care with massive fluids and blood transfusion, peripheral blood stem-cell transplantation, cultured skin-cell grafts, and the administration of cytokines for marrow, the patient was not saved. Restoration of the bone marrow function, prevention of skin fibrosis, radiation lung damage, and repair of gastrointestinal mucosa, and final recovery of the patient were elusive. Abundant personnel and resources were also a prerequisite to allow for the comprehensive and collective intensive care. A further understanding of the effects of high-dose radiation as well as the basic and

  16. Detecting Regional Myocardial Abnormalities in Patients With Wolff-Parkinson-White Syndrome With the Use of ECG-Gated Cardiac MDCT.

    Science.gov (United States)

    Lee, Hye-Jeong; Uhm, Jae-Sun; Joung, Boyoung; Hong, Yoo Jin; Hur, Jin; Choi, Byoung Wook; Kim, Young Jin

    2016-04-01

    Myocardial dyskinesia caused by the accessory pathway and related reversible heart failure have been well documented in echocardiographic studies of pediatric patients with Wolff-Parkinson-White (WPW) syndrome. However, the long-term effects of dyskinesia on the myocardium of adult patients have not been studied in depth. The goal of the present study was to evaluate regional myocardial abnormalities on cardiac CT examinations of adult patients with WPW syndrome. Of 74 patients with WPW syndrome who underwent cardiac CT from January 2006 through December 2013, 58 patients (mean [± SD] age, 52.2 ± 12.7 years), 36 (62.1%) of whom were men, were included in the study after the presence of combined cardiac disease was excluded. Two observers blindly evaluated myocardial thickness and attenuation on cardiac CT scans. On the basis of CT findings, patients were classified as having either normal or abnormal findings. We compared the two groups for other clinical findings, including observations from ECG, echocardiography, and electrophysiologic study. Of the 58 patients studied, 16 patients (27.6%) were found to have myocardial abnormalities (i.e., abnormal wall thinning with or without low attenuation). All abnormal findings corresponded with the location of the accessory pathway. Patients with abnormal findings had statistically significantly decreased left ventricular function, compared with patients with normal findings (p syndrome. These abnormal findings might reflect the long-term effects of dyskinesia, suggesting irreversible myocardial injury that ultimately causes left ventricular dysfunction.

  17. MLPA analysis for a panel of syndromes with mental retardation reveals imbalances in 5.8% of patients with mental retardation and dysmorphic features, including duplications of the Sotos syndrome and Williams-Beuren syndrome regions

    DEFF Research Database (Denmark)

    Kirchhoff, Maria; Bisgaard, Anne-Marie; Bryndorf, Thue

    2007-01-01

    MLPA analysis for a panel of syndromes with mental retardation (MRS-MLPA) was used for investigation of 258 mentally retarded and dysmorphic patients with normal conventional karyotypes (P064 probe set, MRC-Holland, for detection of (micro)deletions associated with 1p36-deletion, Sotos, Williams...... referred with a clinical suspicion of a specific syndrome, which was confirmed in 17 patients (21.3%). The remaining 90 patients were referred because of mental retardation and dysmorphism but without suspicion of a specific syndrome. Seven imbalances, including four duplications, were detected in these 90...

  18. Pericentrin expression in Down's syndrome.

    Science.gov (United States)

    Salemi, Michele; Barone, Concetta; Romano, Carmelo; Salluzzo, Roberto; Caraci, Filippo; Cantarella, Rita Anna; Salluzzo, Maria Grazia; Drago, Filippo; Romano, Corrado; Bosco, Paolo

    2013-11-01

    Down's syndrome (DS) is the most frequent genetic cause of intellectual disability and is a chromosomal abnormality of chromosome 21 trisomy. The pericentrin gene (PCNT) has sequenced in 21q22.3 inside of the minimal critical region for Down's syndrome. Alterations of PCNT gene are associated with dwarfism, cardiomyopathy and other pathologies. In this study, we have evaluated the possible differential expression of PCNT mRNA, by qRT-PCR, in peripheral blood leukocytes of DS subjects compared with the normal population. In the present case-control study, PCNT gene expression was increased by 72.72% in 16 out 22 DS samples compared with normal subjects. Our data suggest that changes in the expression levels of PCNT in DS subjects may be involved into the molecular mechanism of Down's syndrome.

  19. Population - A Critical Factor in the Formation of the Regional System of the Land of the Moţi

    Directory of Open Access Journals (Sweden)

    CRISTIAN NICOLAE BOŢAN

    2009-01-01

    Full Text Available “The Land of the Moţi” is a regional geographical entity where the impact of the anthropic component is essential. If, for a long period, the population has been a cohesion factor in the birth of this regional system, at present, by means of the negative features of the demographical indicators, the population stands out by inducing several elements of high risk. The massive emigration of the population, especially from the areas of high altitude, the gentrification process, the low degree of economic development, are all serious problems which must be on the agenda of the decision-making political factors.

  20. CADM1 is a strong neuroblastoma candidate gene that maps within a 3.72 Mb critical region of loss on 11q23

    International Nuclear Information System (INIS)

    Michels, Evi; Speleman, Frank; Hoebeeck, Jasmien; De Preter, Katleen; Schramm, Alexander; Brichard, Bénédicte; De Paepe, Anne; Eggert, Angelika; Laureys, Geneviève; Vandesompele, Jo

    2008-01-01

    Recurrent loss of part of the long arm of chromosome 11 is a well established hallmark of a subtype of aggressive neuroblastomas. Despite intensive mapping efforts to localize the culprit 11q tumour suppressor gene, this search has been unsuccessful thus far as no sufficiently small critical region could be delineated for selection of candidate genes. To refine the critical region of 11q loss, the chromosome 11 status of 100 primary neuroblastoma tumours and 29 cell lines was analyzed using a BAC array containing a chromosome 11 tiling path. For the genes mapping within our refined region of loss, meta-analysis on published neuroblastoma mRNA gene expression datasets was performed for candidate gene selection. The DNA methylation status of the resulting candidate gene was determined using re-expression experiments by treatment of neuroblastoma cells with the demethylating agent 5-aza-2'-deoxycytidine and bisulphite sequencing. Two small critical regions of loss within 11q23 at chromosomal band 11q23.1-q23.2 (1.79 Mb) and 11q23.2-q23.3 (3.72 Mb) were identified. In a first step towards further selection of candidate neuroblastoma tumour suppressor genes, we performed a meta-analysis on published expression profiles of 692 neuroblastoma tumours. Integration of the resulting candidate gene list with expression data of neuroblastoma progenitor cells pinpointed CADM1 as a compelling candidate gene. Meta-analysis indicated that CADM1 expression has prognostic significance and differential expression for the gene was noted in unfavourable neuroblastoma versus normal neuroblasts. Methylation analysis provided no evidence for a two-hit mechanism in 11q deleted cell lines. Our study puts CADM1 forward as a strong candidate neuroblastoma suppressor gene. Further functional studies are warranted to elucidate the role of CADM1 in neuroblastoma development and to investigate the possibility of CADM1 haploinsufficiency in neuroblastoma

  1. Equivalence of chain conformations in the surface region of a polymer melt and a single Gaussian chain nder critical conditions

    NARCIS (Netherlands)

    Skvortsov, A.M.; Leermakers, F.A.M.; Fleer, G.J.

    2013-01-01

    In the melt polymer conformations are nearly ideal according to Flory's ideality hypothesis. Silberberg generalized this statement for chains in the interfacial region. We check the Silberberg argument by analyzing the conformations of a probe chain end-grafted at a solid surface in a sea of

  2. [Peroral and transdermal application of non-steroidal anti-inflammatory drugs (NSAIDs) for the treatment of regional musculoskeletal pain syndromes].

    Science.gov (United States)

    Hodinka, László; Bálint, Géza; Budai, Erika; Géher, Pál; Papp, Renáta; Somogyi, Péter; Szántó, Sándor; Vereckei, Edit

    2017-12-01

    In this review the available evidences regarding the most frequently applied medication (peroral and transdermal non-steroidal anti-inflammatory agents) for the most frequent musculoskeletal complaints (regional pain syndromes) have been collected for the appropriate medical professionals who are most frequently faced with these conditions (general practitioners, rheumatologists, orthopedics, occupational and sports medicine experts). The special population at risk (with repeated and high energy overuse because of occupational or sport activities) and the pathology of their syndromes are identified. Mode of action, pharmacological properties of the non-steroidal anti-inflammatory drugs and the unwanted effects of their application especially in infants and elderly are highlighted. Recommendations of the general and specific pain management guidelines have been selected and listed in the review. Orv Hetil. 2017; 158(Suppl. 3): 3-30.

  3. Prevalence of metabolic syndrome in Murcia Region, a southern European Mediterranean area with low cardiovascular risk and high obesity

    Directory of Open Access Journals (Sweden)

    Huerta José M

    2011-07-01

    Full Text Available Abstract Background Metabolic syndrome (MS is associated with subsequent appearance of diabetes and cardiovascular disease. As compared to other Spanish regions, Murcia (southern Spain registers increased obesity as well as cardiovascular morbidity and mortality. The aim of this study was to assess the prevalence of MS and its components, awareness of obesity as a health risk and associated lifestyles. Methods A population-based, cross-sectional study was conducted in 2003, covering a sample of 1555 individuals 20 years and over. MS was defined according to the Revised National Cholesterol Education Program Adult Treatment Panel III (R-ATPIII, International Diabetes Federation (IDF and Joint Interim Statement (JIS criteria. Both low (94/80 and high (102/88 waist circumference (WC thresholds were considered. Results Prevalence of MS was 27.2% (95%CI: 25.2-29.2, 32.2% (95%CI: 30.1-34.3 and 33.2% (95%CI: 31.2-35.3 according to the R-ATPIII, IDF and JIS94/80 respectively. It increased with age until reaching 52.6% (R-ATPIII or 60.3% (JIS94/80 among persons aged 70 years and over, and was higher in persons with little or no formal education (51.7% R-ATPIII, 57.3% JIS94/80. The most common risk factors were hypertension (46.6% and central obesity (40.7% and 66.1% according to high and low WC cut-off points respectively. Although most persons were aware that obesity increased health risks, regular exercise was very unusual (13.0% centrally obese, 27.2% non-centrally obese. Adherence to dietary recommendations was similar among centrally obese and non-centrally obese subjects. Conclusions Prevalence of MS is high in our population, is comparable to that found in northern Europe and varies with the definition used. Adherence to preventive recommendations and to adequate weight promotion is very low. In the absence of a specific treatment for MS, integrated intervention based on a sustained increase in physical activity and changes in diet should be

  4. Low-Dose Intravenous Immunoglobulin Treatment for Long-Standing Complex Regional Pain Syndrome: A Randomized Trial.

    Science.gov (United States)

    Goebel, Andreas; Bisla, Jatinder; Carganillo, Roy; Frank, Bernhard; Gupta, Rima; Kelly, Joanna; McCabe, Candy; Murphy, Caroline; Padfield, Nick; Phillips, Ceri; Sanders, Mark; Serpell, Mick; Shenker, Nick; Shoukrey, Karim; Wyatt, Lynne; Ambler, Gareth

    2017-10-03

    Two small trials suggest that low-dose intravenous immunoglobulin (IVIg) may improve the symptoms of complex regional pain syndrome (CRPS), a rare posttraumatic pain condition. To confirm the efficacy of low-dose IVIg compared with placebo in reducing pain during a 6-week period in adult patients who had CRPS from 1 to 5 years. 1:1 parallel, randomized, placebo-controlled, multicenter trial for 6 weeks, with an optional 6-week open extension. Patients were randomly assigned to 1 of 2 study groups between 27 August 2013 and 28 October 2015; the last patient completed follow-up on 21 March 2016. Patients, providers, researchers, and outcome assessors were blinded to treatment assignment. (ISRCTN42179756). 7 secondary and tertiary care pain management centers in the United Kingdom. 111 patients with moderate or severe CRPS of 1 to 5 years' duration. IVIg, 0.5 g/kg of body weight, or visually indistinguishable placebo of 0.1% albumin in saline on days 1 and 22 after randomization. The primary outcome was 24-hour average pain intensity, measured daily between days 6 and 42, on an 11-point (0- to 10-point) rating scale. Secondary outcomes were pain interference and quality of life. The primary analysis sample consisted of 108 eligible patients, 103 of whom had outcome data. Mean (average) pain scores were 6.9 points (SD, 1.5) for placebo and 7.2 points (SD, 1.3) for IVIg. The adjusted difference in means was 0.27 (95% CI, -0.25 to 0.80; P = 0.30), which excluded the prespecified, clinically important difference of -1.2. No statistically significant differences in secondary outcomes were found between the groups. In the open extension, 12 of the 67 patients (18%) who received 2 IVIg infusions had pain reduction of at least 2 points compared with their baseline score. Two patients in the blinded phase (1 in the placebo and 1 in the IVIg group) and 4 in the open IVIg phase had serious events. Results do not apply to patients who have had CRPS for less than 1 year or more

  5. Systemic inflammatory mediators in post-traumatic complex regional pain syndrome (CRPS I) - longitudinal investigations and differences to control groups.

    Science.gov (United States)

    Schinkel, Christian; Scherens, A; Köller, M; Roellecke, G; Muhr, G; Maier, C

    2009-03-17

    The Complex Regional Pain Syndrome I (CRPS I) is a disease that might affect an extremity after trauma or operation. The pathogenesis remains yet unclear. It has clinical signs of severe local inflammation as a result of an exaggerated inflammatory response but neurogenic dysregulation also contributes to it. Some studies investigated the role inflammatory mediators and cytokines; however, few longitudinal studies exist and control groups except healthy controls were not investigated yet. To get further insights into the role of systemic inflammatory mediators in CRPS I, we investigated a variety of pro-, anti-, or neuro-inflammatory mediators such as C-Reactive Protein (CRP), White Blood Cell Count (WBC), Interleukins 4, 6, 8, 10, 11, 12 (p70), Interferon gamma, Tumor-Necrosis-Factor alpha (TNF-a) and its soluble Receptors I/II, soluble Selectins (E,L,P), Substance-P (SP), and Calcitonin Gene-Related Peptide (CGRP) at different time points in venous blood from patients with acute (AC) and chronic (CC) CRPS I, patients with forearm fractures (FR), with neuralgia (NE), and from healthy volunteers (C). No significant changes for serum parameters investigated in CRPS compared to control groups were found except for CC/C (CGRP p = 0.007), FR/C (CGRP p = 0.048) and AC/CC (IL-12 p = 0.02; TNFRI/II p = 0.01; SP p = 0.049). High interindividual variations were observed. No intra- or interindividual correlation of parameters with clinical course (e.g. chronification) or outcome was detectable. Although clinically appearing as inflammation in acute stages, local rather than systemic inflammatory responses seem to be relevant in CRPS. Variable results from different studies might be explained by unpredictable intermittent release of mediators from local inflammatory processes into the blood combined with high interindividual variabilities. A clinically relevant difference to various control groups was not notable in this pilot study. Determination of systemic inflammatory

  6. Systemic inflammatory mediators in post-traumatic Complex Regional Pain Syndrome (CRPS I - longitudinal investigations and differences to control groups

    Directory of Open Access Journals (Sweden)

    Schinkel Ch

    2009-03-01

    Full Text Available Abstract Objectives The Complex Regional Pain Syndrome I (CRPS I is a disease that might affect an extremity after trauma or operation. The pathogenesis remains yet unclear. It has clinical signs of severe local inflammation as a result of an exaggerated inflammatory response but neurogenic dysregulation also contributes to it. Some studies investigated the role inflammatory mediators and cytokines; however, few longitudinal studies exist and control groups except healthy controls were not investigated yet. Methods To get further insights into the role of systemic inflammatory mediators in CRPS I, we investigated a variety of pro-, anti-, or neuro-inflammatory mediators such as C-Reactive Protein (CRP, White Blood Cell Count (WBC, Interleukins 4, 6, 8, 10, 11, 12 (p70, Interferon gamma, Tumor-Necrosis-Factor alpha (TNF-α and its soluble Receptors I/II, soluble Selectins (E, L, P, Substance-P (SP, and Calcitonin Gene-Related Peptide (CGRP at different time points in venous blood from patients with acute (AC and chronic (CC CRPS I, patients with forearm fractures (FR, with neuralgia (NE, and from healthy volunteers (C. Results No significant changes for serum parameters investigated in CRPS compared to control groups were found except for CC/C (CGRP p = 0.007, FR/C (CGRP p = 0.048 and AC/CC (IL-12 p = 0.02; TNFRI/II p = 0.01; SP p = 0.049. High interindividual variations were observed. No intra-or interindividual correlation of parameters with clinical course (e.g. chronification or outcome was detectable. Conclusion Although clinically appearing as inflammation in acute stages, local rather than systemic inflammatory responses seem to be relevant in CRPS. Variable results from different studies might be explained by unpredictable intermittent release of mediators from local inflammatory processes into the blood combined with high interindividual variabilities. A clinically relevant difference to various control groups was not notable in this

  7. Complex regional pain syndrome type I in the upper extremity - how efficient physical therapy and rehabilitation are.

    Science.gov (United States)

    Zečević Luković, Tanja; Ristić, Branko; Jovanović, Zorica; Rančić, Nemanja; Ignjatović Ristić, Dragana; Cuković, Saša

    2012-08-01

    To evaluate the effects of early started combined therapy in Complex Regional Pain Syndrome-1 (CRPS-1) on the upper extremities. The study included 36 patients in the first stadium of CRPS-1 on the upper extremities The mean age of patients was 42.6±14.6, the majority of them (26 of 36) were females. The right side of the upper extremity was affected much more then the left side. They were treated by combined therapy including analgetics, electrotherapy, magneto therapy and kinesitherapy. The average length of observation was 172.1 days (from 90 to 250 days). The average length of treatment was 91.5±42.16 days. Intensity of pain, swelling of the extremity, the change in skin coloration and cutaneous manifestations were assessed three times, at the beginning of the treatment, after 6 weeks and at the end of the treatment. The pain was registered in all patients at visit 1 (average pain intensity was 5.70 ±1.44 on 100 mm visual analogue scale), and it was progressively decreased during the treatment from 3.60±1.22 at the second visit to 0.34±0.68 at the third visit. Vasodilatation was registered in 30 (83.33%) patients and skin temperature asymmetries was found in 21 (58.33%) patients. The difference of size was detected in 30 (83.33%) patients at the first visit compared to four (11.11%) patients at the end of the treatment. There were six (16.66%) patients without swelling at the beginning compared to 26 (72.22%) at the end of the treatment (p less than 0.000). Complete healing was achieved in 32 patients (88.88%). The carefully chosen physical agents in combination with analgesic and non-steroidal anti-inflammatory drugs may benefit in patients with CRPS-1 on the upper extremity if the treatment starts as soon as possible.

  8. Phase analysis of regional and global ventricular contraction patterns in Wolff-Parkinson-White syndrome. Assessment by radionuclide ventriculography

    Energy Technology Data Exchange (ETDEWEB)

    Konishi, Tokuji; Koyama, Takao; Ichikawa, Takehiko and others

    1989-04-01

    Multigated blood pool scintigraphy was performed in 20 normal subjects and 39 patients with various intraventricular conduction abnormalities, including 25 patients with Wolff-Parkinson-White (WPW) syndrome. Cardiac imaging was performed in the modified left anterior oblique, right anterior oblique, and left lateral projections. In WPW syndrome, early contraction sites which were not seen in normal subjects were detected at the ventricular base in phase images. These anomalous early contraction sites disappeared after successful suppression of conduction through an accessory pathway by intravenous procainamide. These sites are believed to correspond to the location of the bundle of Kent and were consistent with the electrocardiographic findings. Phase mapping is a suitable noninvasive method to locate the position of the bundle of Kent and evaluate the ventricular contraction pattern in WPW syndrome and other intraventricular conduction abnormalities. (author).

  9. Critical appraisal of cerebral blood flow measured from brain stem and cerebellar regions after 133 Xe inhalation in humans

    International Nuclear Information System (INIS)

    Juge, O.; Meyer, J.S.; Sakai, F.; Yamaguchi, F.; Yamamoto, M.; Shaw, T.

    1979-01-01

    Validity of regional blood flow (rCBF) measurements recorded over the human posterior fossa after 133Xe inhalation was tested. Recording of counts from both brain stem and cerebellum (BSC) was reproducible and contamination by counts derived from surrounding anatomical structures was low and no greater than that found over hemispheres. BSC flow values showed significant correlation with the state of awareness as judged by clinical and EEG evaluation

  10. Nonmuscle myosin heavy chain IIA is a critical factor contributing to the efficiency of early infection of severe fever with thrombocytopenia syndrome virus.

    Science.gov (United States)

    Sun, Yinyan; Qi, Yonghe; Liu, Chenxuan; Gao, Wenqing; Chen, Pan; Fu, Liran; Peng, Bo; Wang, Haimin; Jing, Zhiyi; Zhong, Guocai; Li, Wenhui

    2014-01-01

    Severe fever with thrombocytopenia syndrome virus (SFTSV) is a novel phlebovirus in the Bunyaviridae family. Most patients infected by SFTSV present with fever and thrombocytopenia, and up to 30% die due to multiple-organ dysfunction. The mechanisms by which SFTSV enters multiple cell types are unknown. SFTSV contains two species of envelope glycoproteins, Gn (44.2 kDa) and Gc (56 kDa), both of which are encoded by the M segment and are cleaved from a precursor polypeptide (about 116 kDa) in the endoplasmic reticulum (ER). Gn fused with an immunoglobulin Fc tag at its C terminus (Gn-Fc) bound to multiple cells susceptible to the infection of SFTSV and blocked viral infection of human umbilical vein endothelial cells (HUVECs). Immunoprecipitation assays following mass spectrometry analysis showed that Gn binds to nonmuscle myosin heavy chain IIA (NMMHC-IIA), a cellular protein with surface expression in multiple cell types. Small interfering RNA (siRNA) knockdown of NMMHC-IIA, but not the closely related NMMHC-IIB or NMMHC-IIC, reduced SFTSV infection, and NMMHC-IIA specific antibody blocked infection by SFTSV but not other control viruses. Overexpression of NMMHC-IIA in HeLa cells, which show limited susceptivity to SFTSV, markedly enhanced SFTSV infection of the cells. These results show that NMMHC-IIA is critical for the cellular entry of SFTSV. As NMMHC-IIA is essential for the normal functions of platelets and human vascular endothelial cells, it is conceivable that NMMHC-IIA directly contributes to the pathogenesis of SFTSV and may be a useful target for antiviral interventions against the viral infection.

  11. Numerical study of criticality of the slab reactors with three regions in one-group transport theory

    International Nuclear Information System (INIS)

    Santos, A. dos.

    1979-01-01

    The criticality of slab reactors consisting of core, blanket, and reflector is studied numerically based on the singular-eigenfunction-expansion method in one-group transport theory. The purpose of this work is three-fold: (1) it is shown that the three-media problem can be converted, using a recently developed method, to a set of regular integral equations for the expansion coefficients, such that numerical solutions can be obtained for the first time based on an exact theory; (2) highly accurate numerical results that can serve as standards of comparison for various approximate methods are reported for representative sets of parameters; and (3) the accuracy of the P sub(N) approximation, one of the more often used methods, is analyzed compared to the exact results [pt

  12. Bisphosphonates Inhibit Pain, Bone Loss, and Inflammation in a Rat Tibia Fracture Model of Complex Regional Pain Syndrome.

    Science.gov (United States)

    Wang, Liping; Guo, Tian-Zhi; Hou, Saiyun; Wei, Tzuping; Li, Wen-Wu; Shi, Xiaoyou; Clark, J David; Kingery, Wade S

    2016-10-01

    Bisphosphonates are used to prevent the bone loss and fractures associated with osteoporosis, bone metastases, multiple myeloma, and osteogenesis deformans. Distal limb fractures cause regional bone loss with cutaneous inflammation and pain in the injured limb that can develop into complex regional pain syndrome (CRPS). Clinical trials have reported that antiresorptive bisphosphonates can prevent fracture-induced bone loss, inhibit serum inflammatory cytokine levels, and alleviate CRPS pain. Previously, we observed that the inhibition of inflammatory cytokines or adaptive immune responses attenuated the development of pain behavior in a rat fracture model of CRPS, and we hypothesized that bisphosphonates could prevent pain behavior, trabecular bone loss, postfracture cutaneous cytokine upregulation, and adaptive immune responses in this CRPS model. Rats underwent tibia fracture and cast immobilization for 4 weeks and were chronically administered either subcutaneously perfused alendronate or oral zoledronate. Behavioral measurements included hindpaw von Frey allodynia, unweighting, warmth, and edema. Bone microarchitecture was measured by microcomputed tomography, and bone cellular activity was evaluated by static and dynamic histomorphometry. Spinal cord Fos immunostaining was performed, and skin cytokine (tumor necrosis factor, interleukin [IL]-1, IL-6) and nerve growth factor (NGF) levels were determined by enzyme immunoassay. Skin and sciatic nerve immunoglobulin levels were determined by enzyme immunoassay. Rats with tibia fractures developed hindpaw allodynia, unweighting, warmth, and edema, increased spinal Fos expression and trabecular bone loss in the lumbar vertebra and bilateral distal femurs as measured by microcomputed tomography, increased trabecular bone resorption and osteoclast surface with decreased bone formation rates, increased cutaneous inflammatory cytokine and NGF expression, and elevated immunocomplex deposition in skin and nerve

  13. Contralateral regional recurrence after elective unilateral neck irradiation in oropharyngeal carcinoma: A literature-based critical review.

    Science.gov (United States)

    Al-Mamgani, Abrahim; van Werkhoven, Erik; Navran, Arash; Karakullukcu, Baris; Hamming-Vrieze, Olga; Machiels, Melanie; van der Velden, Lilly-Ann; Vogel, Wouter V; Klop, W Martin

    2017-09-01

    The head and neck region has rich regional lymphatic network, with a theoretical risk on contralateral metastasis from oropharyngeal cancer (OPC). There is a long-standing convention to irradiate the great majority of these tumors electively to both sides of the neck to reduce the risk of contralateral regional failure (cRF), but this can induce significant toxicity. We aimed to identify patient groups where elective contralateral irradiation may safely be omitted. PubMed and EMBASE were searched for original full-text articles in English with a combination of search terms related to the end points: cRF in OPC primarily treated by radiotherapy only to the ipsilateral neck and identifying predictive factors for increased incidence of cRF. The data from the identified studies were pooled, the incidence of cRF was calculated and the correlation with different predictive factors was investigated. Eleven full-text articles met the inclusion criteria. In these studies, 1116 patients were treated to the ipsilateral neck alone. The mean incidence of cRF was 2.42% (range 0-5.9%, 95% CI 1.6-3.5%). The incidence of cRF correlated only with T-stage (p=0.008), and involvement of midline (p=0.001). However, the significant correlation with T-stage can be explained by the very low incidence of cRF among T1 (0.77%), and disappeared when the incidence of cRF was compared between T2, T3,and T4 (p=0.344). The incidence of cRF in patients with OPC is very low, with involvement of midline providing the most significant prognosticator. These results call for trials on unilateral elective irradiation in selected groups. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Identification of a Major Dimorphic Region in the Functionally Critical N-Terminal ID1 Domain of VAR2CSA

    DEFF Research Database (Denmark)

    Doritchamou, Justin; Sabbagh, Audrey; Jespersen, Jakob S

    2015-01-01

    The VAR2CSA protein of Plasmodium falciparum is transported to and expressed on the infected erythrocyte surface where it plays a key role in placental malaria (PM). It is the current leading candidate for a vaccine to prevent PM. However, the antigenic polymorphism integral to VAR2CSA poses...... a challenge for vaccine development. Based on detailed analysis of polymorphisms in the sequence of its ligand-binding N-terminal region, currently the main focus for vaccine development, we assessed var2csa from parasite isolates infecting pregnant women. The results reveal for the first time the presence...

  15. Differential regional cerebral glucose metabolism in clinical syndromes of frontotemporal lobar degeneration: a study with FDG PET

    International Nuclear Information System (INIS)

    Park, J. M.; Cho, S. S.; Na, D. L.; Lee, K. H.; Choi, Y.; Choe, Y. S.; Kim, B. T.; Kim, S. E.

    2001-01-01

    Frontotemporal lobar degeneration( FTLD) is the third most common dementia, following Alzheimer's disease and Lewy body disease. Four prototypic neurobehavioral syndromes can be produced by FTLD: frontotemporal dementia (FTD), frontotemporal dementia with motor neuron disease (MND), semantic dementia (SD), and progressive aphasia (PA). We investigated patterns of metabolic impairment in patient with FTLD presented with four different clinical syndromes. We analysed glucose metabolic patterns on FDG PET images obtained from 34 patients with a clinical diagnosis of FTLD (19 FTD, 6 MND, 6 SD, and 3 PA, according to a consensus criteria for clinical syndromes associated with FTLD) and 7 age-matched healthy controls using SPM99. Patients with FTD had metabolic deficit in the left frontal cortex and bilateral anterior temporal cortex. Hypometabolism in the bilateral premotor are was shown in patients with MND. Patients with SD had metabolic deficit in the left posterior temporal cortex including Wernicke's area, while hypometabolism in the bilateral inferior frontal gyrus including Broca's area and left angular gyrus was seen in patients with PA. These metabolic patterns were well correlated with clinical features of FTLD syndromes. These data provide a biochemical basis of clinical classification of FTLD. FDG PET may help evaluate and classify patients with FTLD

  16. Deletions at chromosome regions 7q11.23 and 7q36 in a patient with Williams syndrome

    NARCIS (Netherlands)

    Wouters, C. H.; Meijers-Heijboer, H. J.; Eussen, B. J.; van der Heide, A. A.; van Luijk, R. B.; van Drunen, E.; Beverloo, B. B.; Visscher, F.; van Hemel, J. O.

    2001-01-01

    We report on a patient with Williams syndrome and a complex de novo chromosome rearrangement, including microdeletions at 7q11.23 and 7q36 and additional chromosomal material at 7q36. The nature of this additional material was elucidated by spectral karyotyping and first assigned to chromosome 22.

  17. Differential regional cerebral glucose metabolism in clinical syndromes of frontotemporal lobar degeneration: a study with FDG PET

    Energy Technology Data Exchange (ETDEWEB)

    Park, J. M.; Cho, S. S.; Na, D. L.; Lee, K. H.; Choi, Y.; Choe, Y. S.; Kim, B. T.; Kim, S. E. [College of Medicine, Sungkyunkwan Univ., Seoul (Korea, Republic of)

    2001-07-01

    Frontotemporal lobar degeneration( FTLD) is the third most common dementia, following Alzheimer's disease and Lewy body disease. Four prototypic neurobehavioral syndromes can be produced by FTLD: frontotemporal dementia (FTD), frontotemporal dementia with motor neuron disease (MND), semantic dementia (SD), and progressive aphasia (PA). We investigated patterns of metabolic impairment in patient with FTLD presented with four different clinical syndromes. We analysed glucose metabolic patterns on FDG PET images obtained from 34 patients with a clinical diagnosis of FTLD (19 FTD, 6 MND, 6 SD, and 3 PA, according to a consensus criteria for clinical syndromes associated with FTLD) and 7 age-matched healthy controls using SPM99. Patients with FTD had metabolic deficit in the left frontal cortex and bilateral anterior temporal cortex. Hypometabolism in the bilateral premotor are was shown in patients with MND. Patients with SD had metabolic deficit in the left posterior temporal cortex including Wernicke's area, while hypometabolism in the bilateral inferior frontal gyrus including Broca's area and left angular gyrus was seen in patients with PA. These metabolic patterns were well correlated with clinical features of FTLD syndromes. These data provide a biochemical basis of clinical classification of FTLD. FDG PET may help evaluate and classify patients with FTLD.

  18. Application of advanced surface and volumetric NDE methods to the detection of cracks in critical regions of turbine blades

    International Nuclear Information System (INIS)

    Porter, J.P.

    1990-01-01

    Advanced NDE inspection techniques capable of detecting small, yet potentially dangerous cracks in turbine blade tenons, blade tie-wire through-holes, trailing edges, and blade root attachment ends have been devised and developed and are now being applied successfully in the field replacing conventional, less-sensitive methods commonly used for crack detection in these blade elements. Under-shroud lateral cracks in tenons are detected ultrasonically by highangle refracted pulse-echo shear wave and 0-degree pitch-catch longitudinal wave methods. Trailing-edge blade cracks and surface-connected cracks in root attachment ends are detected by high frequency eddy current techniques, typically applied remotely using ports in the turbine housing to gain access to the parts under inspection. Cracks emanating from tie-wire holes in blade upper ends are detected by eddy current inspection, which has been found to be a far more effective methods than either magnetic particle or ultrasonic testing for this application. Root attachment ends of side entry blades are inspected volumetrically by ultrasonics, using proprietary coupling techniques that allow examination of heretofore uninspectable regions of blade attachment hooks, known regions of crack initiation. Techniques developed for this collection of applications are described, and the results of actual field inspections are presented and discussed

  19. POLYCYSTIC OVARY SYNDROME IN ADOLESCENCE

    Directory of Open Access Journals (Sweden)

    Diana Baptista

    2017-02-01

    Conclusion: Identification of adolescents at risk for Polycystic Ovary Syndrome is critical, not only for an appropriate therapeutic approach, but also to prevent co-morbidities associated with the syndrome, including obesity, insulin resistance, dyslipidemia and infertility.

  20. A Retrospective Review of the Use of Regional Citrate Anticoagulation in Continuous Venovenous Hemofiltration for Critically Ill Patients

    Directory of Open Access Journals (Sweden)

    Anne Kit-Hung Leung

    2013-01-01

    Full Text Available Background. The emergence of a commercially prepared citrate solution has revolutionized the use of RCA in the intensive care unit (ICU. The aim of this study was to evaluate the safety profile of a commercially prepared citrate solution. Method. Predilution continuous venovenous hemofiltration (CVVH was performed using Prismocitrate 10/2 at 2500 mL/h and a blood flow rate of 150 mL/min. Calcium chloride solution was infused to maintain ionized calcium within 1.0–1.2 mmol/L. An 8.4% sodium bicarbonate solution was infused separately. Treatment was stopped when the predefined clinical target was reached or the filter clotted. Result. 58 sessions of citrate RCA were analyzed. The median circuit lifetime was 26.0 h (interquartile range IQR 21.2–44.3. The percentage of circuits lasting more than 12 h, 24 h, and 48 h was 94.6%, 58.9%, and 16.1%, respectively. There was no incidence of hypernatremia and median pH was 2.5, only four patients had evidence of citrate accumulation. Conclusion. The commercially prepared citrate solution could be used safely in critically ill patients who required CVVH with no major adverse events.

  1. Concurrent Peripheral Pathologies and Complex Regional Pain Syndrome Type 1 as Contributors to Acute Post-Stroke Shoulder Pain: A Case Report

    Directory of Open Access Journals (Sweden)

    Seyed Majid Akhavan Hejazi

    2012-04-01

    Full Text Available Post-stroke shoulder pain is associated with either a peripheral or central pathology. However, most of the time, it is challenging to establish a cause-and-effect relationship between the suggested pathology and shoulder pain reported. We report a 66 year-old man who developed a right hemiplegic shoulder pain two months post stroke with initial investigations suggestive of peripheral pathologies. Pharmacological and non-pharmacological treatment did not improve his shoulder pain. Later he developed complex regional pain syndrome (CRPS of the right hand and the initial shoulder pain subsequently relieved following resolution of the CRPS.

  2. Refinement of the critical 2p25.3 deletion region: the role of MYT1L in intellectual disability and obesity.

    Science.gov (United States)

    De Rocker, Nina; Vergult, Sarah; Koolen, David; Jacobs, Eva; Hoischen, Alexander; Zeesman, Susan; Bang, Birgitte; Béna, Frédérique; Bockaert, Nele; Bongers, Ernie M; de Ravel, Thomy; Devriendt, Koenraad; Giglio, Sabrina; Faivre, Laurence; Joss, Shelagh; Maas, Saskia; Marle, Nathalie; Novara, Francesca; Nowaczyk, Malgorzata J M; Peeters, Hilde; Polstra, Abeltje; Roelens, Filip; Rosenberg, Carla; Thevenon, Julien; Tümer, Zeynep; Vanhauwaert, Suzanne; Varvagiannis, Konstantinos; Willaert, Andy; Willemsen, Marjolein; Willems, Marjolaine; Zuffardi, Orsetta; Coucke, Paul; Speleman, Frank; Eichler, Evan E; Kleefstra, Tjitske; Menten, Björn

    2015-06-01

    Submicroscopic deletions of chromosome band 2p25.3 are associated with intellectual disability and/or central obesity. Although MYT1L is believed to be a critical gene responsible for intellectual disability, so far no unequivocal data have confirmed this hypothesis. In this study we evaluated a cohort of 22 patients (15 sporadic patients and two families) with a 2p25.3 aberration to further refine the clinical phenotype and to delineate the role of MYT1L in intellectual disability and obesity. In addition, myt1l spatiotemporal expression in zebrafish embryos was analyzed by quantitative polymerase chain reaction and whole-mount in situ hybridization. Complete MYT1L deletion, intragenic deletion, or duplication was observed in all sporadic patients, in addition to two patients with a de novo point mutation in MYT1L. The familial cases comprise a 6-Mb deletion in a father and his three children and a 5' MYT1L overlapping duplication in a father and his two children. Expression analysis in zebrafish embryos shows specific myt1l expression in the developing brain. Our data strongly strengthen the hypothesis that MYT1L is the causal gene for the observed syndromal intellectual disability. Moreover, because 17 patients present with obesity/overweight, haploinsufficiency of MYT1L might predispose to weight problems with childhood onset.Genet Med 17 6, 460-466.

  3. Critical review of the draft Area Recommendation Report and region-to-area screening methodology for the Crystalline Repository Project

    International Nuclear Information System (INIS)

    1986-01-01

    Two documents related to the Crystalline Repository Project have been reviewed. Comments and concerns related to the review of the ''Region-To-Area Screening Methodology'' and the ''Draft Area Recommendation Report'' are presented. These comments will be considered in preparation of the Final Area Recommendation Report, which will serve to formally identify potentially acceptable sites for a second repository in crystalline rock. Following a detailed review of the aforementioned documents, it is concluded that the identification of proposed potentially acceptable sites in the Draft Area Recommendation Report is based on questionable screening methodology and often incomplete data. As a result, ''favorable characteristics'' that are ascribed to each of the proposed potentially acceptable sites are, in many cases, misleading

  4. Integrated GIS and multivariate statistical analysis for regional scale assessment of heavy metal soil contamination: A critical review.

    Science.gov (United States)

    Hou, Deyi; O'Connor, David; Nathanail, Paul; Tian, Li; Ma, Yan

    2017-12-01

    Heavy metal soil contamination is associated with potential toxicity to humans or ecotoxicity. Scholars have increasingly used a combination of geographical information science (GIS) with geostatistical and multivariate statistical analysis techniques to examine the spatial distribution of heavy metals in soils at a regional scale. A review of such studies showed that most soil sampling programs were based on grid patterns and composite sampling methodologies. Many programs intended to characterize various soil types and land use types. The most often used sampling depth intervals were 0-0.10 m, or 0-0.20 m, below surface; and the sampling densities used ranged from 0.0004 to 6.1 samples per km 2 , with a median of 0.4 samples per km 2 . The most widely used spatial interpolators were inverse distance weighted interpolation and ordinary kriging; and the most often used multivariate statistical analysis techniques were principal component analysis and cluster analysis. The review also identified several determining and correlating factors in heavy metal distribution in soils, including soil type, soil pH, soil organic matter, land use type, Fe, Al, and heavy metal concentrations. The major natural and anthropogenic sources of heavy metals were found to derive from lithogenic origin, roadway and transportation, atmospheric deposition, wastewater and runoff from industrial and mining facilities, fertilizer application, livestock manure, and sewage sludge. This review argues that the full potential of integrated GIS and multivariate statistical analysis for assessing heavy metal distribution in soils on a regional scale has not yet been fully realized. It is proposed that future research be conducted to map multivariate results in GIS to pinpoint specific anthropogenic sources, to analyze temporal trends in addition to spatial patterns, to optimize modeling parameters, and to expand the use of different multivariate analysis tools beyond principal component analysis

  5. Identification of the critical depth-of-cut through a 2D image of the cutting region resulting from taper cutting of brittle materials

    Science.gov (United States)

    Gu, Wen; Zhu, Zhiwei; Zhu, Wu-Le; Lu, Leyao; To, Suet; Xiao, Gaobo

    2018-05-01

    An automatic identification method for obtaining the critical depth-of-cut (DoC) of brittle materials with nanometric accuracy and sub-nanometric uncertainty is proposed in this paper. With this method, a two-dimensional (2D) microscopic image of the taper cutting region is captured and further processed by image analysis to extract the margin of generated micro-cracks in the imaging plane. Meanwhile, an analytical model is formulated to describe the theoretical curve of the projected cutting points on the imaging plane with respect to a specified DoC during the whole cutting process. By adopting differential evolution algorithm-based minimization, the critical DoC can be identified by minimizing the deviation between the extracted margin and the theoretical curve. The proposed method is demonstrated through both numerical simulation and experimental analysis. Compared with conventional 2D- and 3D-microscopic-image-based methods, determination of the critical DoC in this study uses the envelope profile rather than the onset point of the generated cracks, providing a more objective approach with smaller uncertainty.

  6. A case report of pseudo grey platelet syndrome with citrate-induced pseudothrombocytopenia: those artifacts may interfere in the platelet numeration and lead to critical misdiagnosis.

    Science.gov (United States)

    Herb, Agathe; Maurer, Maxime; Alamome, Isabelle; Bihl, Pierre-Adrien; Ghiura, Cosmina; Hurstel, Rémy

    2017-08-01

    The pseudo grey platelet syndrome is a rare artifact due to the degranulation of platelets caused, in vitro, by EDTA. This phenomenon is likely to disturb the platelet numeration and it is essential not to mistake it for a grey syndrome platelet, which is a constitutional thrombopathy with macrothrombopenia, in order to avoid specialized tests, or even misdiagnosis. Indeed, these two entities are cytologically alike, as grey platelets are found on the blood smear of a sample collected on EDTA in both cases. We here describe the case of a patient admitted in Colmar's Hospital for a chronic thrombocytopenia, associating both a pseudothrombocytopenia and a pseudo grey platelet syndrome.

  7. Lean techniques to improve the flow of critically ill patients in a health region with its epicenter in the intensive care unit of a reference hospital.

    Science.gov (United States)

    Sirvent, J M; Gil, M; Alvarez, T; Martin, S; Vila, N; Colomer, M; March, E; Loma-Osorio, P; Metje, T

    2016-01-01

    To analyze whether the application of Lean techniques to improve the flow of critically ill patients in a health region with its epicenter in the intensive care unit (ICU) of a reference hospital. Observational study with pre and post intervention analysis. ICU of a reference hospital. We design projects and a value stream map of flow and compared pre and post intervention. We recorded demographic data, patient transfers by EMS for lack of beds and delay times in the discharge from ICU to ward. Multidisciplinary meetings and perform daily visual panel, with high priority ICU discharge. We promote temporary relocation of critically ill patients in other special areas of the hospital. We performed a professional satisfaction questionnaire with pre and post implementation of process. We make a statistical analysis of pre and post-intervention comparisons. We planned for 2013 and progressively implemented in 2014. Analysis of patients entering the critical process flow 1) evaluate patients who must transfer for lack of beds, focusing on a diagnosis: pre 10/22 vs. 3/21 post (P=.045); 2) analysis of time delay in the discharge from the ICU to ward: 360.8±163.9minutes in the first period vs. 276.7±149.5 in the second (P=.036); and 3) personal professional satisfaction questionnaire, with 6.6±1.5 points pre vs. 7.5±1.1 in post (P=.001). Analysis of indicators such as the ICU acquired infections, length of ICU stay, the rate of re-admissions and mortality, with no significant differences between the two periods. The application of Lean techniques in the critically ill process had a positive impact on improving patient flow within the health region, noting a decrease of transfers outside the region due to lack of beds, reduced delayed discharge from ICU to conventional ward and increased satisfaction of ICU professionals. Copyright © 2015 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.

  8. Comparative study of fast critical burner reactors and subcritical accelerator driven systems and the impact on transuranics inventory in a regional fuel cycle

    International Nuclear Information System (INIS)

    Romanello, V.; Salvatores, M.; Schwenk-Ferrero, A.; Gabrielli, F.; Maschek, W.; Vezzoni, B.

    2011-01-01

    Research highlights: → Double-strata fuel cycle has a potential to minimize transuranics mass in Europe. → European Minor Actinides legacy can be reduced down to 0 before the end of century. → 40% higher capacity needed to burn MA for fast critical reactor then for EFIT fleet. → Na cooled fast reactor cores with high content of MA and low CR have been assessed. → Fast critical and ADS-EFIT reactors show comparable MA transmutation performance. - Abstract: In the frame of Partitioning and Transmutation (P and T) strategies, many solutions have been proposed in order to burn transuranics (TRU) discharged from conventional thermal reactors in fast reactor systems. This is due to the favourable feature of neutron fission to capture cross section ratio in a fast neutron spectrum for most TRU. However the majority of studies performed use the Accelerator Driven Systems (ADS), due to their potential flexibility to utilize various fuel types, loaded with significant amounts of TRU having very different Minor Actinides (MA) over Pu ratios. Recently the potential of low conversion ratio critical fast reactors has been rediscovered, with very attractive burning capabilities. In the present paper the burning performances of two systems are directly compared: a sodium cooled critical fast reactor with a low conversion ratio, and the European lead cooled subcritical ADS-EFIT reactor loaded with fertile-free fuel. Comparison is done for characteristics of both the intrinsic core and the regional fuel cycle within a European double-strata scenario. Results of the simulations, obtained by use of French COSI6 code, show comparable performance and confirm that in a double strata fuel cycle the same goals could be achieved by deploying dedicated fast critical or ADS-EFIT type reactors. However the critical fast burner reactor fleet requires ∼30-40% higher installed power then the ADS-EFIT one. Therefore full comparative assessment and ranking can be done only by a

  9. Two-phase flow characteristic of inverted bubbly, slug and annular flow in post-critical heat flux region

    International Nuclear Information System (INIS)

    Ishii, M.; Denten, J.P.

    1988-01-01

    Inverted annular flow can be visualized as a liquid jet-like core surrounded by a vapor annulus. While many analytical and experimental studies of heat transfer in this regime have been performed, there is very little understanding of the basic hydrodynamics of the post-CHF flow field. However, a recent experimental study was done that was able to successfully investigate the effects of various steady-state inlet flow parameters on the post-CHF hydrodynamics of the film boiling of a single phase liquid jet. This study was carried out by means of a visual photographic analysis of an idealized single phase core inverted annular flow initial geometry (single phase liquid jet core surrounded by a coaxial annulus of gas). In order to extend this study, a subsequent flow visualization of an idealized two-phase core inverted annular flow geometry (two-phase central jet core, surrounded by a coaxial annulus of gas) was carried out. The objective of this second experimental study was to investigate the effect of steady-state inlet, pre-CHF two-phase jet core parameters on the hydrodynamics of the post-CHF flow field. In actual film boiling situations, two-phase flows with net positive qualities at the CHF point are encountered. Thus, the focus of the present experimental study was on the inverted bubbly, slug, and annular flow fields in the post dryout film boiling region. Observed post dryout hydrodynamic behavior is reported. A correlation for the axial extent of the transition flow pattern between inverted annular and dispersed droplet flow (the agitated regime) is developed. It is shown to depend strongly on inlet jet core parameters and jet void fraction at the dryout point. 45 refs., 9 figs., 4 tabs

  10. Risk Factors for Post-treatment Complex Regional Pain Syndrome (CRPS): An Analysis of 647 Cases of CRPS from the Danish Patient Compensation Association.

    Science.gov (United States)

    Petersen, Pelle B; Mikkelsen, Kim L; Lauritzen, Jes B; Krogsgaard, Michael R

    2018-03-01

    Complex regional pain syndrome is a challenging condition that includes a broad spectrum of sensory, autonomic, and motor features predominantly in extremities recovering from a trauma. Few large-scale studies have addressed occurrence of and factors associated with complex regional pain syndrome (CRPS) following orthopedic treatment. The present study aimed to identify factors associated with post-treatment development of CRPS. Using the Danish Patient Compensation Association's database, we identified 647 patients claiming post-treatment CRPS between 1992 and 2015. Age, gender, initial diagnosis, treatment, and amount of compensation were extracted. Multivariate logistic regressions were performed to identify variables associated with approval of the claim. For carpal tunnel syndrome (CTS) patients, we registered whether symptoms were bilateral or unilateral and if neurophysiology prior to treatment was pathologic. The following ratios were found: women:men was 4:1, primary diagnosis to the upper limb:lower limb was 2.5:1, and surgical:nonsurgical treatment was 3:1. Mean age was 47.5 ± 13.7 years, and no intergender difference was detected. Antebrachial fracture (23%) and CTS (9%) were the most common primary conditions. Surgical treatment was associated with approval of the claim (odds ratio 3.5, 95% confidence interval 2.3 to 5.3; P CRPS patients. In CTS patients developing CRPS, normal neurophysiological examination findings were common, and it could be suspected that these patients were suffering from an pre-clinical stage of CRPS, not CTS. © 2017 World Institute of Pain.