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Sample records for gm1 gangliosidosis patients

  1. Genetics Home Reference: GM1 gangliosidosis

    MedlinePlus

    ... more common in particular ethnic groups? Genetic Changes Mutations in the GLB1 gene cause GM1 gangliosidosis . The ... normal functioning of nerve cells in the brain. Mutations in the GLB1 gene reduce or eliminate the ...

  2. Chemical chaperone therapy for brain pathology in GM1-gangliosidosis

    PubMed Central

    Matsuda, Junichiro; Suzuki, Osamu; Oshima, Akihiro; Yamamoto, Yoshie; Noguchi, Akira; Takimoto, Kazuhiro; Itoh, Masayuki; Matsuzaki, Yuji; Yasuda, Yosuke; Ogawa, Seiichiro; Sakata, Yuko; Nanba, Eiji; Higaki, Katsumi; Ogawa, Yoshimi; Tominaga, Lika; Ohno, Kousaku; Iwasaki, Hiroyuki; Watanabe, Hiroshi; Brady, Roscoe O.; Suzuki, Yoshiyuki

    2003-01-01

    We synthesized a galactose derivative, N-octyl-4-epi-?-valienamine (NOEV), for a molecular therapy (chemical chaperone therapy) of a human neurogenetic disease, ?-galactosidosis (GM1-gangliosidosis and Morquio B disease). It is a potent inhibitor of lysosomal ?-galactosidase in vitro. Addition of NOEV in the culture medium restored mutant enzyme activity in cultured human or murine fibroblasts at low intracellular concentrations, resulting in a marked decrease of intracellular substrate storage. Short-term oral administration of NOEV to a model mouse of juvenile GM1-gangliosidosis, expressing a mutant enzyme protein R201C, resulted in significant enhancement of the enzyme activity in the brain and other tissues. Immunohistochemical stain revealed a decrease in the amount of GM1 and GA1 in neuronal cells in the fronto-temporal cerebral cortex and brainstem. However, mass biochemical analysis did not show the substrate reduction observed histochemically in these limited areas in the brain probably because of the brief duration of this investigation. Chemical chaperone therapy may be useful for certain patients with ?-galactosidosis and potentially other lysosomal storage diseases with central nervous system involvement. PMID:14676316

  3. Candidate molecules for chemical chaperone therapy of GM1-gangliosidosis.

    PubMed

    Higaki, Katsumi; Ninomiya, Haruaki; Suzuki, Yoshiyuki; Nanba, Eiji

    2013-09-01

    A growing body of evidence suggests that misfolding of a mutant protein followed by its aggregation or premature degradation in the endoplasmic reticulum is one of the main mechanisms that underlie inherited neurodegenerative diseases, including lysosomal storage diseases. Chemical or pharmacological chaperones are small molecules that bind to and stabilize mutant lysosomal enzyme proteins in the endoplasmic reticulum. A number of chaperone compounds for lysosomal hydrolases have been identified in the last decade. They have gained attention because they can be orally administrated, and also because they can penetrate the blood-brain barrier. In this article, we describe two chaperone candidates for the treatment of GM1-gangliosidosis. We also discuss the future direction of this strategy targeting other lysosomal storage diseases as well as protein misfolding diseases in general. PMID:24024947

  4. Feline GM1 gangliosidosis: characterization of the residual liver acid beta-galactosidase.

    PubMed Central

    Holmes, E W; O'Brien, J S

    1978-01-01

    The residual liver acid beta-galactosidase (beta-gal) activity from a case of feline GM1 gangliosidosis was partially purified and characterized with respect to its pH optimum, kinetic properties, thermostability, isoelectric point, molecular weight, and antigenicity. In comparison to the normal enzyme, the mutant enzyme had the same pH optima for the three substrates tested, a reduced Km for 4-methylumbelliferyl-beta-gal, elevated Km's for GM1 and asialofetuin (ASF), and increased thermolability. In addition, the mutant beta-gal had a higher isoelectric point, a reduced molecular weight, and appeared to be antigenically different from normal. The results suggest that the mutation in the Birmingham GM1 cat is structural and that the residual enzyme activity is a structurally altered acid beta-gal. The apparent lack of antigenic identity between the mutant and normal enzymes, in contrast to the situation in many human GM1 patients, is most unusual. PMID:83795

  5. A novel human model of the neurodegenerative disease GM1 gangliosidosis using induced pluripotent stem cells demonstrates inflammasome activation.

    PubMed

    Son, Mi-Young; Kwak, Jae Eun; Seol, Binna; Lee, Da Yong; Jeon, Hyejin; Cho, Yee Sook

    2015-09-01

    GM1 gangliosidosis (GM1) is an inherited neurodegenerative disorder caused by mutations in the lysosomal β-galactosidase (β-gal) gene. Insufficient β-gal activity leads to abnormal accumulation of GM1 gangliosides in tissues, particularly in the central nervous system, resulting in progressive neurodegeneration. Here, we report an in vitro human GM1 model, based on induced pluripotent stem cell (iPSC) technology. Neural progenitor cells differentiated from GM1 patient-derived iPSCs (GM1-NPCs) recapitulated the biochemical and molecular phenotypes of GM1, including defective β-gal activity and increased lysosomes. Importantly, the characterization of GM1-NPCs established that GM1 is significantly associated with the activation of inflammasomes, which play a critical role in the pathogenesis of various neurodegenerative diseases. Specific inflammasome inhibitors potently alleviated the disease-related phenotypes of GM1-NPCs in vitro and in vivo. Our data demonstrate that GM1-NPCs are a valuable in vitro human GM1 model and suggest that inflammasome activation is a novel target pathway for GM1 drug development. PMID:25925601

  6. Dog GM1 gangliosidosis: characterization of the residual liver acid beta-galactosidase.

    PubMed Central

    Rittmann, L S; Tennant, L L; O'Brien, J S

    1980-01-01

    The residual liver acid beta-galactosidase activity from the first documented case of GM1 gangliosidosis in dogs was partially purified and characterized with respect to kinetic properties, thermostability, isoelectric point, molecular weight, and antigenicity. The GM1 dog liver beta-galactosidase appears to be identical with the normal dog liver enzyme in all properties examined. The canine disease is strikingly different from the human disease in the amount of enzyme that is present in the tissue. Unlike the human disease, in which normal amounts of catalytically defective beta-galactosidase are present, in dog GM1 gangliosidosis, only 1% of normal beta-galactosidase protein is detectable. PMID:6778205

  7. MRI/MRS as a surrogate marker for clinical progression in GM1 gangliosidosis.

    PubMed

    Regier, Debra S; Kwon, Hyuk Joon; Johnston, Jean; Golas, Gretchen; Yang, Sandra; Wiggs, Edythe; Latour, Yvonne; Thomas, Sarah; Portner, Cindy; Adams, David; Vezina, Gilbert; Baker, Eva H; Tifft, Cynthia J

    2016-03-01

    Background GM1 gangliosidosis is a lysosomal storage disorder caused by mutations in GLB1, encoding β-galactosidase. The range of severity is from type I infantile disease, lethal in early childhood, to type III adult onset, resulting in gradually progressive neurological symtpoms in adulthood. The intermediate group of patients has been recently classified as having type II late infantile subtype with onset of symptoms at one to three years of age or type II juvenile subtype with symptom onset at 2-10 years. To characterize disease severity and progression, six Late infantile and nine juvenile patients were evaluated using magnetic resonance imaging (MRI), and MR spectroscopy (MRS). Since difficulties with ambulation (gross motor function) and speech (expressive language) are often the first reported symptoms in type II GM1, patients were also scored in these domains. Deterioration of expressive language and ambulation was more rapid in the late infantile patients. Fourteen MRI scans in six Late infantile patients identified progressive atrophy in the cerebrum and cerebellum. Twenty-six MRI scans in nine juvenile patients revealed greater variability in extent and progression of atrophy. Quantitative MRS demonstrated a deficit of N-acetylaspartate in both the late infantile and juvenile patients with greater in the late infantile patients. This correlates with clinical measures of ambulation and expressive language. The two subtypes of type II GM1 gangliosidosis have different clinical trajectories. MRI scoring, quantitative MRS and brain volume correlate with clinical disease progression and may serve as important minimally-invasive outcome measures for clinical trials. © 2015 Wiley Periodicals, Inc. PMID:26646981

  8. Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases.

    PubMed Central

    Yoshida, K; Oshima, A; Shimmoto, M; Fukuhara, Y; Sakuraba, H; Yanagisawa, N; Suzuki, Y

    1991-01-01

    Molecular analysis of the human beta-galactosidase gene revealed six different mutations in 10 of 11 Japanese GM1-gangliosidosis patients. They were the only abnormalities in each allele examined in this study. A 165-nucleotide duplication (positions 1103-1267) was found in two infantile patients, producing an abnormally large mRNA; one patient was probably a homozygote, and the other was a heterozygote of this mutation. The other two infantile patients had different mutations; a 123 Gly(GGG)----Arg(AGG) mutation in one patient and a 316 Tyr(TAT)----Cys(TGT) mutation in the other. A 201 Arg(CGC)----Cys(TGC) mutation, eliminating a BspMI site, was detected in a late-infantile/juvenile patient; the restriction-site analysis of amplified genomic DNA confirmed his heterozygosity for this mutation. A 51 Ile(ATC)----Thr(ACC) mutation was found in all five adult/chronic patients examined in this study. It created a SauI site, and restriction-site analysis confirmed that four patients were homozygous mutants. The other was a compound heterozygote for this mutation and another 457 Arg(CGA)----Gln(CAA) mutation. These mutant genes expressed markedly decreased or completely deficient enzyme activities in beta-galactosidase-deficient human fibroblasts transformed by adenovirus-SV40 recombinants. We conclude that gene mutations are heterogeneous in GM1-gangliosidosis but that the 51 Ile(ATC)----Thr(ACC) mutation is common among the Japanese adult/chronic cases. Genotype-phenotype correlations in GM1-gangliosidosis are briefly discussed. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 PMID:1907800

  9. N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis.

    PubMed

    Kasperzyk, Julie L; El-Abbadi, Mohga M; Hauser, Eric C; D'Azzo, Alessandra; Platt, Frances M; Seyfried, Thomas N

    2004-05-01

    GM1 gangliosidosis is a glycosphingolipid (GSL) lysosomal storage disease caused by a genetic deficiency of acid beta-galactosidase (beta-gal), the enzyme that catabolyzes GM1 within lysosomes. Accumulation of GM1 and its asialo form (GA1) occurs primarily in the brain, leading to progressive neurodegeneration and brain dysfunction. Substrate reduction therapy aims to decrease the rate of GSL biosynthesis to counterbalance the impaired rate of catabolism. The imino sugar N-butyldeoxygalactonojirimycin (NB-DGJ) is a competitive inhibitor of the ceramide-specific glucosyltransferase that catalyzes the first step in GSL biosynthesis. Neonatal C57BL/6J (B6) and beta-gal knockout (-/-) mice were injected daily from post-natal day 2 (p-2) to p-5 with either vehicle or NB-DGJ at 600 mg or 1200 mg/kg body weight. These drug concentrations significantly reduced total brain ganglioside and GM1 content in the B6 and the beta-gal (-/-) mice. Drug treatment had no significant effect on viability, body weight, brain weight, or brain water content in the B6 and beta-gal (-/-) mice. Significant elevations in neutral lipids (GA1, ceramide, and sphingomyelin) were observed in the NB-DGJ-treated beta-gal (-/-) mice, but were not associated with adverse effects. Also, NB-DGJ treatment of B6 and beta-gal (-/-) mice from p-2 to p-5 had no subsequent effect on brain ganglioside content at p-21. Our results show that NB-DGJ is effective in reducing total brain ganglioside and GM1 content at early neonatal ages. These findings suggest that substrate reduction therapy using NB-DGJ may be an effective early intervention for GM1 gangliosidosis and possibly other GSL lysosomal storage diseases. PMID:15086521

  10. GM1-gangliosidosis in American black bears: clinical, pathological, biochemical and molecular genetic characterization.

    PubMed

    Muthupalani, Sureshkumar; Torres, Paola A; Wang, Betty C; Zeng, Bai Jin; Eaton, Samuel; Erdelyi, Ildiko; Ducore, Rebecca; Maganti, Rajanikarath; Keating, John; Perry, Bain J; Tseng, Florina S; Waliszewski, Nicole; Pokras, Mark; Causey, Robert; Seger, Rita; March, Philip; Tidwell, Amy; Pfannl, Rolf; Seyfried, Thomas; Kolodny, Edwin H; Alroy, Joseph

    2014-04-01

    G(M1)-gangliosidosis is a rare progressive neurodegenerative disorder due to an autosomal recessively inherited deficiency of lysosomal β-galactosidase. We have identified seven American black bears (Ursus americanus) found in the Northeast United States suffering from G(M1)-gangliosidosis. This report describes the clinical features, brain MRI, and morphologic, biochemical and molecular genetic findings in the affected bears. Brain lipids were compared with those in the brain of a G(M1)-mouse. The bears presented at ages 10-14 months in poor clinical condition, lethargic, tremulous and ataxic. They continued to decline and were humanely euthanized. The T(2)-weighted MR images of the brain of one bear disclosed white matter hyperintensity. Morphological studies of the brain from five of the bears revealed enlarged neurons with foamy cytoplasm containing granules. Axonal spheroids were present in white matter. Electron microscopic examination revealed lamellated membrane structures within neurons. Cytoplasmic vacuoles were found in the liver, kidneys and chondrocytes and foamy macrophages within the lungs. Acid β-galactosidase activity in cultured skin fibroblasts was only 1-2% of control values. In the brain, ganglioside-bound sialic acid was increased more than 2-fold with G(M1)-ganglioside predominating. G(A1) content was also increased whereas cerebrosides and sulfatides were markedly decreased. The distribution of gangliosides was similar to that in the G(M1)-mouse brain, but the loss of myelin lipids was greater in the brain of the affected bear than in the brain of the G(M1) mouse. Isolated full-length cDNA of the black bear GLB1 gene revealed 86% homology to its human counterpart in nucleotide sequence and 82% in amino acid sequence. GLB1 cDNA from liver tissue of an affected bear contained a homozygous recessive T(1042) to C transition inducing a Tyr348 to His mutation (Y348H) within a highly conserved region of the GLB1 gene. The coincidence of several black bears with G(M1)-gangliosidosis in the same geographic area suggests increased frequency of a founder mutation in this animal population. PMID:24581871

  11. Myelin Abnormalities in the Optic and Sciatic Nerves in Mice With GM1-Gangliosidosis

    PubMed Central

    Heinecke, Karie A.; Luoma, Adrienne; d’Azzo, Alessandra; Kirschner, Daniel A.

    2015-01-01

    GM1-gangliosidosis is a glycosphingolipid lysosomal storage disease involving accumulation of GM1 and its asialo form (GA1) primarily in the brain. Thin-layer chromatography and X-ray diffraction were used to analyze the lipid content/composition and the myelin structure of the optic and sciatic nerves from 7- and 10-month old β-galactosidase (β-gal) +/? and β-gal −/− mice, a model of GM1gangliosidosis. Optic nerve weight was lower in the β-gal −/− mice than in unaffected β-gal +/? mice, but no difference was seen in sciatic nerve weight. The levels of GM1 and GA1 were significantly increased in both the optic nerve and sciatic nerve of the β-gal −/− mice. The content of myelin-enriched cerebrosides, sulfatides, and plasmalogen ethanolamines was significantly lower in optic nerve of β-gal −/− mice than in β-gal +/? mice; however, cholesteryl esters were enriched in the β-gal −/− mice. No major abnormalities in these lipids were detected in the sciatic nerve of the β-gal −/− mice. The abnormalities in GM1 and myelin lipids in optic nerve of β-gal −/− mice correlated with a reduction in the relative amount of myelin and periodicity in fresh nerve. By contrast, the relative amount of myelin and periodicity in the sciatic nerves from control and β-gal −/− mice were indistinguishable, suggesting minimal pathological involvement in sciatic nerve. Our results indicate that the greater neurochemical pathology observed in the optic nerve than in the sciatic nerve of β-gal −/− mice is likely due to the greater glycolipid storage in optic nerve. PMID:25694553

  12. A Bicyclic 1-Deoxygalactonojirimycin Derivative as a Novel Pharmacological Chaperone for GM1 Gangliosidosis

    PubMed Central

    Takai, Tomoko; Higaki, Katsumi; Aguilar-Moncayo, Matilde; Mena-Barragán, Teresa; Hirano, Yuki; Yura, Kei; Yu, Liang; Ninomiya, Haruaki; García-Moreno, M. Isabel; Sakakibara, Yasubumi; Ohno, Kousaku; Nanba, Eiji; Ortiz Mellet, Carmen; García Fernández, José M.; Suzuki, Yoshiyuki

    2013-01-01

    Lysosomal β-galactosidase (β-Gal) deficiency causes a group of disorders that include neuronopathic GM1 gangliosidosis and non-neuronopathic Morquio B disease. We have previously proposed the use of small molecule ligands of β-Gal as pharmacological chaperones (PCs) for the treatment of GM1 gangliosidosis brain pathology. Although it is still under development, PC therapy has yielded promising preclinical results in several lysosomal diseases. In this study, we evaluated the effect of bicyclic 1-deoxygalactonojirimycin (DGJ) derivative of the sp2-iminosugar type, namely 5N,6S-(N′-butyliminomethylidene)-6-thio-1- deoxygalactonojirimycin (6S-NBI-DGJ), as a novel PC for human mutant β-Gal. In vitro, 6S-NBI-DGJ had the ability to inhibit the activity of human β-Gal in a competitive manner and was able to protect this enzyme from heat-induced degradation. Computational analysis supported that the rigid glycone bicyclic core of 6S-NBI-DGJ binds to the active site of the enzyme, with the aglycone N′-butyl substituent, in a precise E-orientation, located at a hydrophobic region nearby. Chaperone potential profiling indicated significant increases of enzyme activity in 24 of 88 β-Gal mutants, including four common mutations. Finally, oral administration of 6S-NBI-DGJ ameliorated the brain pathology of GM1 gangliosidosis model mice. These results suggest that 6S-NBI-DGJ is a novel PC that may be effective on a broad range of β-Gal mutants. PMID:23337983

  13. A bicyclic 1-deoxygalactonojirimycin derivative as a novel pharmacological chaperone for GM1 gangliosidosis.

    PubMed

    Takai, Tomoko; Higaki, Katsumi; Aguilar-Moncayo, Matilde; Mena-Barragán, Teresa; Hirano, Yuki; Yura, Kei; Yu, Liang; Ninomiya, Haruaki; García-Moreno, M Isabel; Sakakibara, Yasubumi; Ohno, Kousaku; Nanba, Eiji; Ortiz Mellet, Carmen; García Fernández, José M; Suzuki, Yoshiyuki

    2013-03-01

    Lysosomal β-galactosidase (β-Gal) deficiency causes a group of disorders that include neuronopathic GM1 gangliosidosis and non-neuronopathic Morquio B disease. We have previously proposed the use of small molecule ligands of β-Gal as pharmacological chaperones (PCs) for the treatment of GM1 gangliosidosis brain pathology. Although it is still under development, PC therapy has yielded promising preclinical results in several lysosomal diseases. In this study, we evaluated the effect of bicyclic 1-deoxygalactonojirimycin (DGJ) derivative of the sp(2)-iminosugar type, namely 5N,6S-(N'-butyliminomethylidene)-6-thio-1- deoxygalactonojirimycin (6S-NBI-DGJ), as a novel PC for human mutant β-Gal. In vitro, 6S-NBI-DGJ had the ability to inhibit the activity of human β-Gal in a competitive manner and was able to protect this enzyme from heat-induced degradation. Computational analysis supported that the rigid glycone bicyclic core of 6S-NBI-DGJ binds to the active site of the enzyme, with the aglycone N'-butyl substituent, in a precise E-orientation, located at a hydrophobic region nearby. Chaperone potential profiling indicated significant increases of enzyme activity in 24 of 88 β-Gal mutants, including four common mutations. Finally, oral administration of 6S-NBI-DGJ ameliorated the brain pathology of GM1 gangliosidosis model mice. These results suggest that 6S-NBI-DGJ is a novel PC that may be effective on a broad range of β-Gal mutants. PMID:23337983

  14. Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice.

    PubMed

    Kasperzyk, J L; d'Azzo, A; Platt, F M; Alroy, J; Seyfried, T N

    2005-04-01

    II3NeuAc-GgOse4Cer (GM1) gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid beta-galactosidase (beta-gal), resulting in the accumulation of ganglioside GM1 and its asialo derivative GgOse4Cer (GA1) in the central nervous system, primarily in the brain. In this study, we investigated the effects of N-butyldeoxygalacto-nojirimycin (N B-DGJ), an imino sugar that inhibits ganglioside biosynthesis, in normal C57BL/6J mice and in beta-gal knockout (beta-gal-/-) mice from postnatal day 9 (p-9) to p-15. This is a period of active cerebellar development and central nervous system (CNS) myelinogenesis in the mouse and would be comparable to late-stage embryonic and early neonatal development in humans. N B-DGJ significantly reduced total ganglioside and GM1 content in cerebrum-brainstem (C-BS) and in cerebellum of normal and beta-gal-/- mice. N B-DGJ had no adverse effects on body weight or C-BS/cerebellar weight, water content, or thickness of the external cerebellar granule cell layer. Sphingomyelin was increased in C-BS and cerebellum, but no changes were found for cerebroside (a myelin-enriched glycosphingolipid), neutral phospholipids, or GA1 in the treated mice. Our findings indicate that the effects of N B-DGJ in the postnatal CNS are largely specific to gangliosides and suggest that N B-DGJ may be an effective early intervention therapy for GM1 gangliosidosis and other ganglioside storage disorders. PMID:15687347

  15. Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis.

    PubMed

    Elliot-Smith, Elena; Speak, Anneliese O; Lloyd-Evans, Emyr; Smith, David A; van der Spoel, Aarnoud C; Jeyakumar, Mylvaganam; Butters, Terry D; Dwek, Raymond A; d'Azzo, Alessandra; Platt, Frances M

    2008-06-01

    GM1 gangliosidosis is an inherited neurodegenerative disorder caused by lysosomal beta-galactosidase deficiency, resulting in the storage of GM1 and GA1, primarily in the central nervous system. This disease typically afflicts infants and young children and there is currently no effective therapy. Substrate reduction therapy (SRT) could be of potential benefit. The imino sugars N-butyldeoxynojirimycin (NB-DNJ, miglustat, Zavesca) and N-butyldeoxygalactonojirimycin (NB-DGJ) used for SRT inhibit glucosylceramide synthase (GlcCerS) that catalyses the first committed step in glycosphingolipid biosynthesis. We have compared the efficacy and tolerability of NB-DNJ and NB-DGJ in the beta-galactosidase knockout mouse. NB-DGJ was better tolerated than NB-DNJ, due to intrinsic gastrointestinal tract dysfunction that was exacerbated by NB-DNJ. However, functional improvement was greatest with NB-DNJ treatment which may potentially be caused by novel anti-inflammatory properties of NB-DNJ. PMID:18387328

  16. AAV-Mediated Gene Delivery in Adult GM1-Gangliosidosis Mice Corrects Lysosomal Storage in CNS and Improves Survival

    PubMed Central

    Baek, Rena C.; Broekman, Marike L. D.; Leroy, Stanley G.; Tierney, Laryssa A.; Sandberg, Michael A.; d'Azzo, Alessandra; Seyfried, Thomas N.; Sena-Esteves, Miguel

    2010-01-01

    Background GM1-gangliosidosis is a glycosphingolipid (GSL) lysosomal storage disease caused by a genetic deficiency of acid β-galactosidase (βgal), which results in the accumulation of GM1-ganglioside and its asialo-form (GA1) primarily in the CNS. Age of onset ranges from infancy to adulthood, and excessive ganglioside accumulation produces progressive neurodegeneration and psychomotor retardation in humans. Currently, there are no effective therapies for the treatment of GM1-gangliosidosis. Methodology/Principal Findings In this study we examined the effect of thalamic infusion of AAV2/1-βgal vector in adult GM1 mice on enzyme distribution, activity, and GSL content in the CNS, motor behavior, and survival. Six to eight week-old GM1 mice received bilateral injections of AAV vector in the thalamus, or thalamus and deep cerebellar nuclei (DCN) with pre-determined endpoints at 1 and 4 months post-injection, and the humane endpoint, or 52 weeks of age. Enzyme activity was elevated throughout the CNS of AAV-treated GM1 mice and GSL storage nearly normalized in most structures analyzed, except in the spinal cord which showed ∼50% reduction compared to age-matched untreated GM1 mice spinal cord. Survival was significantly longer in AAV-treated GM1 mice (52 wks) than in untreated mice. However the motor performance of AAV-treated GM1 mice declined over time at a rate similar to that observed in untreated GM1 mice. Conclusions/Significance Our studies show that the AAV-modified thalamus can be used as a ‘built-in’ central node network for widespread distribution of lysosomal enzymes in the mouse cerebrum. In addition, this study indicates that thalamic delivery of AAV vectors should be combined with additional targets to supply the cerebellum and spinal cord with therapeutic levels of enzyme necessary to achieve complete correction of the neurological phenotype in GM1 mice. PMID:20976108

  17. Enzyme replacement for GM1-gangliosidosis: Uptake, lysosomal activation, and cellular disease correction using a novel β-galactosidase:RTB lectin fusion.

    PubMed

    Condori, Jose; Acosta, Walter; Ayala, Jorge; Katta, Varun; Flory, Ashley; Martin, Reid; Radin, Jonathan; Cramer, Carole L; Radin, David N

    2016-02-01

    New enzyme delivery technologies are required for treatment of lysosomal storage disorders with significant pathologies associated with the so-called "hard-to-treat" tissues and organs. Genetic deficiencies in the GLB1 gene encoding acid β-galactosidase lead to GM1-gangliosidosis or Morquio B, lysosomal diseases with predominant disease manifestation associated with the central nervous system or skeletal system, respectively. Current lysosomal ERTs are delivered into cells based on receptor-mediated endocytosis and do not effectively address several hard-to-treat organs including those critical for GM1-gangliosidosis patients. Lectins provide alternative cell-uptake mechanisms based on adsorptive-mediated endocytosis and thus may provide unique biodistribution for lysosomal disease therapeutics. In the current study, genetic fusions of the plant galactose/galactosamine-binding lectin, RTB, and the human acid β-galactosidase enzyme were produced using a plant-based bioproduction platform. β-gal:RTB and RTB:β-gal fusion products retained both lectin activity and β-galactosidase activity. Purified proteins representing both fusion orientations were efficiently taken up into GM1 patient fibroblasts and mediated the reduction of GM1 ganglioside substrate with activities matching mammalian cell-derived β-galactosidase. In contrast, plant-derived β-gal alone was enzymatically active but did not mediate uptake or correction indicating the need for either lectin-based (plant product) or mannose-6-phosphate-based (mammalian product) delivery. Native β-galactosidase undergoes catalytic activation (cleavage within the C-terminal region) in lysosomes and is stabilized by association with protective protein/cathepsin A. Enzymatic activity and lysosomal protein processing of the RTB fusions were assessed following internalization into GM1 fibroblasts. Within 1-4h, both β-gal:RTB and RTB:β-gal were processed to the ~64kDa "activated" β-gal form; the RTB lectin was cleaved and rapidly degraded. The activated β-gal was still detected at 48h suggesting interactions with protective protein/cathepsin A. Uptake-saturation analyses indicated that the RTB adsorptive-mediated mechanisms of β-gal:RTB supported significantly greater accumulation of β-galactose activity in fibroblasts compared to the receptor-mediated mechanisms of the mammalian cell-derived β-gal. These data demonstrate that plant-made β-gal:RTB functions as an effective replacement enzyme for GM1-gangliosidosis - delivering enzyme into cells, enabling essential lysosomal processing, and mediating disease substrate clearance at the cellular level. RTB provides novel uptake behaviors and thus may provide new receptor-independent strategies that could broadly impact lysosomal disease treatments. PMID:26766614

  18. Evaluation of N-nonyl-deoxygalactonojirimycin as a pharmacological chaperone for human GM1 gangliosidosis leads to identification of a feline model suitable for testing enzyme enhancement therapy

    PubMed Central

    Rigat, Brigitte A.; Tropak, Michael B.; Buttner, Justin; Crushell, Ellen; Benedict, Daphne; Callahan, John W.; Martin, Douglas R.; Mahuran, Don J.

    2012-01-01

    Deficiencies of lysosomal β-D-galactosidase can result in GM1 gangliosidosis, a severe neurodegenerative disease characterized by massive neuronal storage of GM1 ganglioside in the brain. Currently there are no available therapies that can even slow the progression of this disease. Enzyme enhancement therapy utilizes small molecules that can often cross the blood brain barrier, but are also often competitive inhibitors of their target enzyme. It is a promising new approach for treating diseases, often caused by missense mutations, associated with dramatically reduced levels of functionally folded enzyme. Despite a number of positive reports based on assays performed with patient cells, skepticism persists that an inhibitor-based treatment can increase mutant enzyme activity in vivo. To date no appropriate animal model, i.e., one that recapitulates a responsive human genotype and clinical phenotype, has been reported that could be used to validate enzyme enhancement therapy. In this report, we identify a novel enzyme enhancement-agent, N-nonyl-deoxygalactonojirimycin, that enhances the mutant β-galactosidase activity in the lysosomes of a number of patient cell lines containing a variety of missense mutations. We then demonstrate that treatment of cells from a previously described, naturally occurring feline model (that biochemically, clinically and molecularly closely mimics GM1 gangliosidosis in humans) with this molecule, results in a robust enhancement of their mutant lysosomal β-galactosidase activity. These data indicate that the feline model could be used to validate this therapeutic approach and determine the relationship between the disease stage at which this therapy is initiated and the maximum clinical benefits obtainable. PMID:22784478

  19. Crystal structure of human β-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases.

    PubMed

    Ohto, Umeharu; Usui, Kimihito; Ochi, Toshinari; Yuki, Kenjiro; Satow, Yoshinori; Shimizu, Toshiyuki

    2012-01-13

    G(M1) gangliosidosis and Morquio B are autosomal recessive lysosomal storage diseases associated with a neurodegenerative disorder or dwarfism and skeletal abnormalities, respectively. These diseases are caused by deficiencies in the lysosomal enzyme β-d-galactosidase (β-Gal), which lead to accumulations of the β-Gal substrates, G(M1) ganglioside, and keratan sulfate. β-Gal is an exoglycosidase that catalyzes the hydrolysis of terminal β-linked galactose residues. This study shows the crystal structures of human β-Gal in complex with its catalytic product galactose or with its inhibitor 1-deoxygalactonojirimycin. Human β-Gal is composed of a catalytic TIM barrel domain followed by β-domain 1 and β-domain 2. To gain structural insight into the molecular defects of β-Gal in the above diseases, the disease-causing mutations were mapped onto the three-dimensional structure. Finally, the possible causes of the diseases are discussed. PMID:22128166

  20. Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.

    PubMed

    Sánchez-Fernández, Elena M; García Fernández, José M; Mellet, Carmen Ortiz

    2016-04-12

    Lysosomal storage disorders (LSDs) are often caused by mutations that destabilize native folding and impair the trafficking of enzymes, leading to premature endoplasmic reticulum (ER)-associated degradation, deficiencies of specific hydrolytic functions and aberrant storage of metabolites in the lysosomes. Enzyme replacement therapy (ERT) and substrate reduction therapy (SRT) are available for a few of these conditions, but most remain orphan. A main difficulty is that virtually all LSDs involve neurological decline and neither proteins nor the current SRT drugs can cross the blood-brain barrier. Twenty years ago a new therapeutic paradigm better suited for neuropathic LSDs was launched, namely pharmacological chaperone (PC) therapy. PCs are small molecules capable of binding to the mutant protein at the ER, inducing proper folding, restoring trafficking and increasing enzyme activity and substrate processing in the lysosome. In many LSDs the mutated protein is a glycosidase and the accumulated substrate is an oligo- or polysaccharide or a glycoconjugate, e.g. a glycosphingolipid. Although it might appear counterintuitive, substrate analogues (glycomimetics) behaving as competitive glycosidase inhibitors are good candidates to perform PC tasks. The advancements in the knowledge of the molecular basis of LSDs, including enzyme structures, binding modes, trafficking pathways and substrate processing mechanisms, have been put forward to optimize PC selectivity and efficacy. Moreover, the chemical versatility of glycomimetics and the variety of structures at hand allow simultaneous optimization of chaperone and pharmacokinetic properties. In this Feature Article we review the advancements made in this field in the last few years and the future outlook through the lessons taught by three archetypical LSDs: Gaucher disease, GM1-gangliosidosis and Fabry disease. PMID:27043200

  1. Synthesis of C-5a-chain extended derivatives of 4-epi-isofagomine: Powerful ?-galactosidase inhibitors and low concentration activators of GM1-gangliosidosis-related human lysosomal ?-galactosidase.

    PubMed

    Thonhofer, Martin; Weber, Patrick; Santana, Andres Gonzalez; Fischer, Roland; Pabst, Bettina M; Paschke, Eduard; Schalli, Michael; Sttz, Arnold E; Tschernutter, Marion; Windischhofer, Werner; Withers, Stephen G

    2016-03-01

    From an easily available partially protected formal derivative of 1-deoxymannojirimycin, by hydroxymethyl chain-branching and further elaboration, lipophilic analogs of the powerful ?-d-galactosidase inhibitor 4-epi-isofagomine have become available. New compounds exhibit improved inhibitory activities comparable to benchmark compound NOEV (N-octyl-epi-valienamine) and may serve as leads towards improved and more selective pharmacological chaperones for GM1-gangliosidosis. PMID:26838810

  2. Mutations in acid [beta]-galactosidase cause GM[sub 1]-gangliosidosis in American patients

    SciTech Connect

    Boustany, R.M.; Qian, W.H. ); Suzuki, K. )

    1993-10-01

    The authors describe four new mutations in the [beta]-galactosidase gene. These are the first mutations causing infantile and juvenile GM[sub 1]-gangliosidosis to be described in American patients. Cell lines from two patients with juvenile and from six patients with infantile GM[sub 1]-gangliosidosis were analyzed. Northern blot analysis showed the acid [beta]-galactosidase message to be of normal size and quantity in two juvenile and four infantile cases and of normal size but reduced quantity in two infantile cases. The mutations are distinct from the Japanese mutations. All are point mutations leading to amino acid substitutions: Lys[sup 577] [yields] Arg, Arg[sup 590] [yields] His, and Glu[sup 632] [yields] Gly. The fourth mutation, Arg[sup 208] [yields] Cys, accounts for 10 of 16 possible alleles. Two infantile cases from Puerto Rico of Spanish ancestry are homozygous for this mutation, suggesting that this allele may have come to South America and North America via Puerto Rico. That these mutations cause clinical disease was confirmed by marked reduction in catalytic activity of the mutant proteins in the Cos-1 cell expression system. 12 refs., 5 figs., 2 tabs.

  3. High-throughput imaging method for direct assessment of GM1 ganglioside levels in mammalian cells.

    PubMed

    Acosta, Walter; Martin, Reid; Radin, David N; Cramer, Carole L

    2016-03-01

    GM1-gangliosidosis is an inherited autosomal recessive disorder caused by mutations in the gene GLB1, which encodes acid β-galactosidase (β-gal). The lack of activity in this lysosomal enzyme leads to accumulation of GM1 gangliosides (GM1) in cells. We have developed a high-content-imaging method to assess GM1 levels in fibroblasts that can be used to evaluate substrate reduction in treated GLB1(-/-) cells [1]. This assay allows fluorescent quantification in a multi-well system which generates unbiased and statistically significant data. Fluorescently labeled Cholera Toxin B subunit (CTXB), which specifically binds to GM1 gangliosides, was used to detect in situ GM1 levels in a fixed monolayer of fibroblasts. This sensitive, rapid, and inexpensive method facilitates in vitro drug screening in a format that allows a high number of replicates using low working volumes. PMID:26958633

  4. High-throughput imaging method for direct assessment of GM1 ganglioside levels in mammalian cells

    PubMed Central

    Acosta, Walter; Martin, Reid; Radin, David N.; Cramer, Carole L.

    2016-01-01

    GM1-gangliosidosis is an inherited autosomal recessive disorder caused by mutations in the gene GLB1, which encodes acid β-galactosidase (β-gal). The lack of activity in this lysosomal enzyme leads to accumulation of GM1 gangliosides (GM1) in cells. We have developed a high-content-imaging method to assess GM1 levels in fibroblasts that can be used to evaluate substrate reduction in treated GLB1−/− cells [1]. This assay allows fluorescent quantification in a multi-well system which generates unbiased and statistically significant data. Fluorescently labeled Cholera Toxin B subunit (CTXB), which specifically binds to GM1 gangliosides, was used to detect in situ GM1 levels in a fixed monolayer of fibroblasts. This sensitive, rapid, and inexpensive method facilitates in vitro drug screening in a format that allows a high number of replicates using low working volumes. PMID:26958633

  5. Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant

    SciTech Connect

    Schepers, U.; Glombitza, G.; Lemm, T.

    1996-11-01

    Lysosomal degradation of ganglioside GM2 by {beta}-hexosaminidase A (hex A) requires the presence of the GM2 activator protein (GM2AP) as an essential cofactor. A deficiency of the GM2 activator causes the AB variant of GM2 gangliosidosis, a recessively inherited disorder characterized by excessive neuronal accumulation of GM2 and related glycolipids. Two novel mutations in the GM2 activator gene (GM2A) have been identified by the reverse-transcriptase-PCR method - a three-base deletion, AAG{sup 262-264}, resulting in a deletion of Lys{sup 88}, and a single-base deletion, A{sup 410}, that causes a frameshift. The latter results in substitution of 33 amino acids and the loss of another 24 amino acid residues. Both patients are homoallelic for their respective mutations inherited from their parents, who are heteroallelic at the GM2A locus. Although the cultured fibroblasts of both patients produce normal levels of activator mRNA, they lack a lysosomal form of GM2AP. Pulse/chase labeling of cultured fibroblasts of the patients, in presence and absence of brefeldin A, indicates a premature degradation of both-mutant and truncated-GM2APs in the endoplasmic reticulum or Golgi. These results were supported by in vitro translation experiments and expression of the mutated proteins. When the mutated GM2APs were expressed in Escherichia coli, both mature GM2AP forms turned proved to exhibit only residual activities in an in vitro assay. 26 refs., 7 figs.

  6. [Generalized GM1 gangliosidosis. Report of a case and review of the literature].

    PubMed

    Pérez, R; Cuesta, J M; Haro de los Monteros, N; Hernando Mayor y Montes, J C

    1984-05-01

    Authors present the case of a child, daughter of non related parents with neurologic progressive affectation, retina and visceral implication with certain pseudogargolic clinical aspect without near familiar antecedents suggestive of this disease. Biochemistry and histologic studies revealed a B-galactosidase enzyme deficiency and lipidic intracellular increase in different viscera. Biochemistry, hystological and clinical aspects exposed are fundamentally differential of another causes of pseudo-Hurler syndrome. PMID:6433763

  7. Deficiency of ganglioside GM1 correlates with Parkinson's disease in mice and humans.

    PubMed

    Wu, Gusheng; Lu, Zi-Hua; Kulkarni, Neil; Ledeen, Robert W

    2012-10-01

    Several studies have successfully employed GM1 ganglioside to treat animal models of Parkinson's disease (PD), suggesting involvement of this ganglioside in PD etiology. We recently demonstrated that genetically engineered mice (B4galnt1(-/-) ) devoid of GM1 acquire characteristic symptoms of this disorder, including motor impairment, depletion of striatal dopamine, selective loss of tyrosine hydroxylase-expressing neurons, and aggregation of α-synuclein. The present study demonstrates similar symptoms in heterozygous mice (HTs) that express only partial GM1 deficiency. Symptoms were alleviated by administration of L-dopa or LIGA-20, a membrane-permeable analog of GM1 that penetrates the blood-brain barrier and accesses intracellular compartments. Immunohistochemical analysis of paraffin sections from PD patients revealed significant GM1 deficiency in nigral dopaminergic neurons compared with age-matched controls. This was comparable to the GM1 deficiency of HT mice and suggests that GM1 deficiency may be a contributing factor to idiopathic PD. We propose that HT mice with partial GM1 deficiency constitute an especially useful model for PD, reflecting the actual pathophysiology of this disorder. The results point to membrane-permeable analogs of GM1 as holding promise as a form of GM1 replacement therapy. PMID:22714832

  8. Binding of immunoglobulin G antibodies in Guillain-Barré syndrome sera to a mixture of GM1 and a phospholipid: possible clinical implications.

    PubMed

    Kusunoki, Susumu; Morita, Daiji; Ohminami, Shinya; Hitoshi, Seiji; Kanazawa, Ichiro

    2003-03-01

    Anti-GM1 immunoglobulin G (IgG) antibodies are frequently present in sera from patients with Guillain-Barré syndrome (GBS). A previous report on a patient who had a neuropathy with immunoglobulin M (IgM) M-protein binding to a conformational epitope formed by phosphatidic acid (PA) and gangliosides prompted us to investigate the binding of IgG antibodies in GBS sera to a mixture of GM1 and PA (GM1/PA). Of 121 GBS patients, 32 had anti-GM1 IgG antibodies. All 32 also had antibody activity against GM1/PA. Twenty-five (78%) of 32 patients had greater activity against GM1/PA than against GM1 alone. Twelve patients who had no anti-GM1 IgG antibodies had IgG antibody activity against GM1/PA. No GBS patient had IgG antibody against PA alone. In contrast, two rabbit anti-GM1 antisera had greater activity against GM1 alone than against GM1/PA. IgG antibody with greater binding activity against a mixture of GM1 and a phospholipid than against GM1 alone may have an important role in the pathogenesis of GBS and has implications for diagnosis. PMID:12635116

  9. An open-label Phase I/II clinical trial of pyrimethamine for the treatment of patients affected with chronic GM2 gangliosidosis (Tay-Sachs or Sandhoff variants).

    PubMed

    Clarke, Joe T R; Mahuran, Don J; Sathe, Swati; Kolodny, Edwin H; Rigat, Brigitte A; Raiman, Julian A; Tropak, Michael B

    2011-01-01

    Late-onset GM2 gangliosidosis is an autosomal recessive, neurodegenerative, lysosomal storage disease, caused by deficiency of ß-hexosaminidase A (Hex A), resulting from mutations in the HEXA (Tay-Sachs variant) or the HEXB (Sandhoff variant) genes. The enzyme deficiency in many patients with juvenile or adult onset forms of the disease results from the production of an unstable protein, which becomes targeted for premature degradation by the quality control system of the smooth endoplasmic reticulum and is not transported to lysosomes. In vitro studies have shown that many mutations in either the α or β subunit of Hex A can be partially rescued, i.e. enhanced levels of both enzyme protein and activity in lysosomes, following the growth of patient cells in the presence of the drug, pyrimethamine. The objectives of the present clinical trial were to establish the tolerability and efficacy of the treatment of late-onset GM2 gangliosidosis patients with escalating doses of pyrimethamine, to a maximum of 100 mg per day, administered orally in a single daily dose, over a 16-week period . The primary objective, tolerability, was assessed by regular clinical examinations, along with a panel of hematologic and biochemical studies. Although clinical efficacy could not be assessed in this short trial, treatment efficacy was evaluated by repeated measurements of leukocyte Hex A activity, expressed relative to the activity of lysosomal ß-glucuronidase. A total of 11 patients were enrolled, 8 males and 3 females, aged 23 to 50 years. One subject failed the initial screen, another was omitted from analysis because of the large number of protocol violations, and a third was withdrawn very early as a result of adverse events which were not drug-related. For the remaining 8 subjects, up to a 4-fold enhancement of Hex A activity at doses of 50 mg per day or less was observed. Additionally marked individual variations in the pharmacokinetics of the drug among the patients were noted. However, the study also found that significant side effects were experienced by most patients at or above 75 mg pyrimethamine per day. We concluded that pyrimethamine treatment enhances leukocyte Hex A activity in patients with late-onset GM2 gangliosidosis at doses lower than those associated with unacceptable side effects. Further plans are underway to extend these trials and to develop methods to assess clinical efficacy. PMID:20926324

  10. Asialo GM1 is a receptor for Pseudomonas aeruginosa adherence to regenerating respiratory epithelial cells.

    PubMed Central

    de Bentzmann, S; Roger, P; Dupuit, F; Bajolet-Laudinat, O; Fuchey, C; Plotkowski, M C; Puchelle, E

    1996-01-01

    We investigated the implication of asialo GM1 as an epithelial receptor in the increased Pseudomonas aeruginosa affinity for regenerating respiratory epithelial cells from cystic fibrosis (CF) and non-CF patients. Human respiratory epithelial cells were obtained from nasal polyps of non-CF subjects and of CF patients homozygous for the delta F 508 transmembrane conductance regulator protein (CFTR) mutation and cultured according to the explant-outgrowth model. At the periphery of the outgrowth, regenerating respiratory epithelial cells spreading over the collagen I matrix with lamellipodia were observed, characteristic of respiratory epithelial wound repair after injury. P aeruginosa adherence to regenerating respiratory epithelial cells was found to be significantly greater in the delta F 508 homozygous CF group than in the non-CF group (P < 0.001). In vitro competitive binding inhibition assays performed with rabbit polyclonal antibody against asialo GM1 demonstrated that blocking asialo GM1 reduces P. aeruginosa adherence to regenerating respiratory epithelial cells in delta F 508 homozygous cultures (P < 0.001) as well as in non-CF cultures (P < 0.001). Blocking of asialo GM1 was significantly more efficient in CF patients than in non-CF subjects (P < 0.05). Distribution of asialo GM1 as determined by preembedding labelling and immunoelectron microscopy clearly demonstrated the specific apical membrane expression of asialo GM1 by regenerating respiratory epithelial cells, whereas other cell phenotypes did not apically express asialo GM1. These results demonstrate that (i) asialo GM1 is an apical membrane receptor for P. aeruginosa expressed at the surface of CF and non-CF regenerating respiratory epithelial cells and (ii) asialo GM1 is specifically recovered in regenerating respiratory epithelium. These results suggest that in CF, epithelial repair represents the major event which exposes asialo GM1 for P. aeruginosa adherence. PMID:8613364

  11. Mutations in the lysosomal [beta]-galactosidase gene that cause the adult form of GMI gangliosidosis

    SciTech Connect

    Chakraborty, S.; Rafi, M.A.; Wenger, D.A. )

    1994-06-01

    Three adult patients with acid-galactosidase deficiency/GM1 gangliosidosis who were from two unrelated families of Scandinavian descent were found to share a common point mutation in the coding region of the corresponding gene. The patients share common clinical features, including early dysarthria, mild ataxia, and bone abnormalities. When cDNA from the two patients in family 1 was PCR amplified and sequenced, most (39/41) of the clones showed a C-to-T transition (C[yields]T) at nucleotide 245 (counting from the initiation codon). This mutation changes the codon for the Thr(ACG) to Met(ATG). Mutant and normal sequences were also found in that position in genomic DNA, indicating the presence of another mutant allele. Genomic DNA from the patient in family 2 revealed the same point mutation in one allele. It was determined that in each family only the father carried the C[yields]T mutation. Expression studies showed that this mutation produced 3%-4% of [beta]-galactosidase activity, confirming its deleterious effects. The cDNA clones from the patients in family 1 that did not contain the C[yields]T revealed a 20-bp insertion of intronic sequence between nucleotides 75 and 76, the location of the first intron. Further analysis showed the insertion of a T near the 5[prime] splice donor site which led to the use of a cryptic splice site. It appears that the C[yields]T mutation results in enough functional enzyme to produce a mild adult form of the disease, even in the presence of a second mutation that likely produces nonfunctional enzyme. 31 refs., 7 figs., 1 tab.

  12. GM1 ganglioside and Alzheimer's disease.

    PubMed

    Yanagisawa, Katsuhiko

    2015-05-01

    Assembly and deposition of amyloid ß-protein (Aß) is an invariable and fundamental event in the pathological process of Alzheimer's disease (AD). To decipher the AD pathogenesis and also to develop disease-modifying drugs for AD, clarification of the molecular mechanism underlying the Aß assembly into amyloid fibrils in the brain has been a crucial issue. GM1-ganglioside-bound Aß (GAß), with unique molecular characteristics such as having an altered conformation and the capability to accelerate Aß assembly, was discovered in an autopsied brain showing early pathological changes of AD in 1995. On the basis of these findings, it was hypothesized that GAß is an endogenous seed for amyloid fibril formation in the AD brain. A body of evidence that supports this GAß hypothesis has been growing over this past 20 years. In this article, seminal GAß studies that have been carried out to date, including recent ones using unique animal models, are reviewed. PMID:25903682

  13. GM1 and GM2 gangliosides: recent developments.

    PubMed

    Bisel, Blaine; Pavone, Francesco S; Calamai, Martino

    2014-03-01

    GM1 and GM2 gangliosides are important components of the cell membrane and play an integral role in cell signaling and metabolism. In this conceptual overview, we discuss recent developments in our understanding of the basic biological functions of GM1 and GM2 and their involvement in several diseases. In addition to a well-established spectrum of disorders known as gangliosidoses, such as Tay-Sachs disease, more and more evidence points at an involvement of GM1 in Alzheimer's and Parkinson's diseases. New emerging methodologies spanning from single-molecule imaging in vivo to simulations in silico have complemented standard studies based on ganglioside extraction. PMID:25372744

  14. GM1 Ganglioside: Past Studies and Future Potential.

    PubMed

    Aureli, Massimo; Mauri, Laura; Ciampa, Maria Grazia; Prinetti, Alessandro; Toffano, Gino; Secchieri, Cynthia; Sonnino, Sandro

    2016-04-01

    Gangliosides (sialic acid-containing glycosphingolipids) are abundant in neurons of all animal species and play important roles in many cell physiological processes, including differentiation, memory control, cell signaling, neuronal protection, neuronal recovery, and apoptosis. Gangliosides also function as anchors or entry points for various toxins, bacteria, viruses, and autoantibodies. GM1, a ganglioside component of mammalian brains, is present mainly in neurons. GM1 is one of the best studied gangliosides, and our understanding of its properties is extensive. Simple and rapid procedures are available for preparation of GM1 as a natural compound on a large scale, or as a derivative containing an isotopic radionuclide or a specific probe. Great research interest in the properties of GM1 arose from the discovery in the early 1970s of its role as receptor for the bacterial toxin responsible for cholera pathogenesis. PMID:25762012

  15. Lipopolysaccharides from Campylobacter jejuni O:41 Strains Associated with Guillain-Barré Syndrome Exhibit Mimicry of GM1 Ganglioside

    PubMed Central

    Prendergast, Martina M.; Lastovica, Albert J.; Moran, Anthony P.

    1998-01-01

    Three Campylobacter jejuni, biotype 2, serotype O:41 strains that were isolated from patients who developed Guillain-Barré syndrome (GBS) and one C. jejuni isolate from a patient who developed enteritis only were examined. The aim of the study was to determine the structure of the core oligosaccharide (OS) of the lipopolysaccharide (LPS) of C. jejuni serotype O:41, a serotype rarely associated with the development of GBS, and to determine if the LPS shares similar epitopes with any of the major human gangliosides. Electrophoretic analysis with silver staining or immunoblotting demonstrated that the strains had LPS profiles characteristic of low-molecular-weight LPS. Colorimetric analysis detected N-acetylneuraminic (sialic) acid in the core OSs of all the strains. Thin-layer chromatography with immunostaining showed that antisera raised against the GBS strains reacted with the GM1 ganglioside, suggesting that C. jejuni serotype O:41 LPSs and the GM1 ganglioside have similar epitopes. Furthermore, polyclonal anti-GM1 and anti-asialoGM1 antibodies cross-reacted with each C. jejuni O:41 LPS tested, suggesting that the serotype O:41 core OS has a GM1- and asialoGM1-like structure. LPSs extracted from C. jejuni serostrains O:2, O:3, and O:19 were also used in the study. Cholera toxin (a GM1 ligand) and peanut agglutinin (a Galβ1–3GalNAc ligand) recognized all serotype O:41 LPSs and the serostrain O:2 LPS. Immunoadsorption results confirmed GM1 relatedness. Moreover, the core OS was isolated from a GBS-associated C. jejuni O:41 LPS by gel permeation chromatography. An analysis by gas-liquid chromatography (GLC), GLC-mass spectrometry, and nuclear magnetic resonance showed the core OS of one of the C. jejuni O:41 GBS isolates to have a tetrasaccharide structure consistent with GM1 mimicry. PMID:9673245

  16. Biochemical characterization of GM1 micelles-Amphotericin B interaction.

    PubMed

    Leonhard, Victoria; Alasino, Roxana V; Bianco, Ismael D; Garro, Ariel G; Heredia, Valeria; Beltramo, Dante M

    2015-01-01

    In this work a thorough characterization of the GM1 micelle-Amphotericin B (AmB) interaction was performed. The micelle formation as well as the drug loading occurs spontaneously, although influenced by the physicochemical conditions, pH and temperature. The chromatographic profile of GM1-AmB complexes at different molar ratios shows the existence of two populations. The differential absorbance of GM1, monomeric and aggregate AmB, allowed us to discriminate the presence of all of them in both fractions. Thus, we noted that at higher proportion of AmB in the complex, increases the larger population which is composed mainly of aggregated AmB. The physical behavior of these micelles shows that both GM1- AmB complexes were stable in solution for at least 30 days. However upon freeze-thawing or lyophilization-solubilization cycles, only the smallest population, enriched in monomeric AmB, showed a complete solubilization. In vitro, GM1-AmB micelles were significantly less toxic on cultured cells than other commercial micellar formulations as Fungizone, but had a similar behavior to liposomal formulations as Ambisome. Regarding the antifungal activity of the new formulation, it was very similar to that of other formulations. The characterization of these GM1-AmB complexes is discussed as a potential new formulation able to improve the antifungal therapeutic efficiency of AmB. PMID:25772153

  17. Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells.

    PubMed Central

    Saiman, L; Prince, A

    1993-01-01

    The basis for the unique association of Pseudomonas aeruginosa and the cystic fibrosis (CF) lung has remained obscure despite major advances in the understanding of the molecular genetic cause of this disease. There is evidence to suggest that abnormalities in CF transmembrane conductance regulator function result in alterations in the glycosylation of epithelial components. The number of asialoGM1 residues, as representative of a class of glycolipids which contain a GalNAc beta 1-4Gal sequence for P. aeruginosa attachment, was quantified by flow cytometric studies of respiratory epithelial cells in primary culture from both CF patients and normal subjects. Superficial asialoGM1 was detected on 12% of the CF cells as compared with 2.9% of the cells from normal control subjects (P = 0.03, chi 2 = 4.73), and more asialoGM1 residues were exposed on CF cells after modification by P. aeruginosa exoproducts. AsialoGM1, but not the sialylated glycolipid GM1, was demonstrated to be a receptor for 125I-labeled P. aeruginosa pilin, a major adhesin for this organism, and exogenous asialoGM1 was found to competitively inhibit P. aeruginosa adherence to epithelial cells, thus, confirming the biological role of the asialoGM1 receptor. Quantitative and qualitative differences in the sialylation of superficial glycolipids in CF epithelial cells may directly contribute to the colonization of the CF lung by P. aeruginosa. Images PMID:8104958

  18. Alzheimer's disease is associated with disordered localization of ganglioside GM1 molecular species in the human dentate gyrus.

    PubMed

    Hirano-Sakamaki, Wakako; Sugiyama, Eiji; Hayasaka, Takahiro; Ravid, Rivka; Setou, Mitsutoshi; Taki, Takao

    2015-11-30

    Alzheimer's disease (AD) is a progressive dementia associated with loss of memory and cognitive dysfunction. In a previous study, we demonstrated a decrease in b-series gangliosides along with a change in ganglioside molecular species in the hippocampal grey matter of patients with AD. The present study demonstrates the use of imaging mass spectrometry for analyzing the spatial arrangement of ganglioside GM1 (GM1) molecular species in the hippocampus. In AD patients, we found a decrease in the ratio of GM1(d20:1/C18:0) to GM1 d18:1/C18:0) in the outer molecular layer (ML) of the dentate gyrus. Because the outer ML is the region of main input into the hippocampus, our findings may have a direct relationship to the mechanism of dysfunction in AD. PMID:26484596

  19. [Serum IgG antibodies to GD1a and GM1 gangliosides in elderly people].

    PubMed

    Kolyovska, V

    2016-01-01

    Nowadays, the percentage of elderly people in society grows. Good nutrition and medical care help older people to have a normal life over 80 to 90 years. In the last ten years it is of critical importance to establish the clinical significance of serum IgG anti-GD1a and anti-GM1 ganglioside antibodies as potential biomarkers for neuronal damage in neurodegenerative diseases and immune-mediated neuropathies and demyelination. In the current study, the diagnostic values of IgG anti-GD1a and anti-GM1 antibodies were determined by the ELISA method in serum samples of 18 elderly patients (71-91 years). Significantly elevated serum IgG anti-GD1a and anti-GM1 antibodies titers were detected only in patients over 80 years. These data suggest that the immune-mediated neuropathies, neurodegeneration and demyelination in healthy elderly occur after 80 years old. Therefore, IgG anti-GD1a and anti-GM1 antibodies can serve as biomarkers, showing the nervous system dysfunction. PMID:26973195

  20. THE EFFECT OF MONOSIALOGANGLYOSIDE (GM-1) ADMINISTRATION IN SPINAL CORD INJURY

    PubMed Central

    BARROS, TARCÍSIO ELOY PESSOA; ARAUJO, FERNANDO FLORES DE; HIGINO, LUCAS DA PAZ; MARCON, RAPHAEL MARTUS; CRISTANTE, ALEXANDRE FOGAÇA

    2016-01-01

    ABSTRACT Objective: To evaluate the effect of monosialoganglioside (GM-1) in spinal cord trauma patients seen in our service who have not been treated with methylprednisolone. Methods: Thirty patients with acute spinal cord trauma were randomly divided into two groups. In Group 1, patients received 200 mg GM-1 in the initial assessment and thereafter received 100 mg intravenous per day for 30 days and Group 2 (control) received saline. Patients were evaluated periodically (at 6 weeks, 6 months, one year and two years), using a standardized neurological assessment of the American Spinal Injury Association / International Spinal Cord Society. Results: The comparative statistical analysis of motor indices, sensitive indices for pain and touch according to the standardization of ASIA / ISCOS showed that the assessments at 6 weeks, 6 months and 2 years, GM-Group 1 patients had higher rates than the control group regarding sensitivity to pain and touch, with no statistically significant difference from the motor index. Conclusion: The functional assessment showed improvement in the sensitive indices of patients treated with GM1 after post-traumatic spinal cord injury compared to patients who received placebo. Level of Evidence IV, Prospective Case Studies Series. PMID:27217811

  1. Individual Restriction Of Fine Specificity Variability In Anti-GM1 IgG Antibodies Associated With Guillain-Barré Syndrome

    PubMed Central

    Lardone, Ricardo D.; Yuki, Nobuhiro; Irazoqui, Fernando J.; Nores, Gustavo A.

    2016-01-01

    Elevated titers of serum antibodies against GM1 ganglioside are associated with a variety of autoimmune neuropathies. Much evidence indicates these autoantibodies play a primary role in the disease processes, but the mechanism for their appearance is unclear. We studied the fine specificity of anti-GM1 antibodies of the IgG isotype present in sera from patients with Guillain-Barré syndrome (GBS), using thin-layer chromatogram-immunostaining of GM1, asialo-GM1 (GA1), GD1b and GM1-derivatives with small modifications on the oligosaccharide moiety. We were able to distinguish populations of antibodies with different fine specificity. Remarkably, individual patients presented only one or two of them, and different patients had different populations. This restriction in the variability of antibody populations suggests that the appearance of the anti-GM1 antibodies is a random process involving restricted populations of lymphocytes. With the origin of disease-associated anti-GM1 antibodies as a context, this finding could provide explanation for the “host susceptibility factor” observed in GBS following enteritis with GM1 oligosaccharide-carrying strains of Campylobacter jejuni. PMID:26818965

  2. Lo/Ld phase coexistence modulation induced by GM1.

    PubMed

    Puff, Nicolas; Watanabe, Chiho; Seigneuret, Michel; Angelova, Miglena I; Staneva, Galya

    2014-08-01

    Lipid rafts are assumed to undergo biologically important size-modulations from nanorafts to microrafts. Due to the complexity of cellular membranes, model systems become important tools, especially for the investigation of the factors affecting "raft-like" Lo domain size and the search for Lo nanodomains as precursors in Lo microdomain formation. Because lipid compositional change is the primary mechanism by which a cell can alter membrane phase behavior, we studied the effect of the ganglioside GM1 concentration on the Lo/Ld lateral phase separation in PC/SM/Chol/GM1 bilayers. GM1 above 1mol % abolishes the formation of the micrometer-scale Lo domains observed in GUVs. However, the apparently homogeneous phase observed in optical microscopy corresponds in fact, within a certain temperature range, to a Lo/Ld lateral phase separation taking place below the optical resolution. This nanoscale phase separation is revealed by fluorescence spectroscopy, including C12NBD-PC self-quenching and Laurdan GP measurements, and is supported by Gaussian spectral decomposition analysis. The temperature of formation of nanoscale Lo phase domains over an Ld phase is determined, and is shifted to higher values when the GM1 content increases. A "morphological" phase diagram could be made, and it displays three regions corresponding respectively to Lo/Ld micrometric phase separation, Lo/Ld nanometric phase separation, and a homogeneous Ld phase. We therefore show that a lipid only-based mechanism is able to control the existence and the sizes of phase-separated membrane domains. GM1 could act on the line tension, "arresting" domain growth and thereby stabilizing Lo nanodomains. PMID:24835016

  3. Intraventricular Sialidase Administration Enhances GM1 Ganglioside Expression and Is Partially Neuroprotective in a Mouse Model of Parkinson’s Disease

    PubMed Central

    Schneider, Jay S.; Seyfried, Thomas N.; Choi, Hyo-S.; Kidd, Sarah K.

    2015-01-01

    Background Preclinical and clinical studies have previously shown that systemic administration of GM1 ganglioside has neuroprotective and neurorestorative properties in Parkinson’s disease (PD) models and in PD patients. However, the clinical development of GM1 for PD has been hampered by its animal origin (GM1 used in previous studies was extracted from bovine brains), limited bioavailability, and limited blood brain barrier penetrance following systemic administration. Objective To assess an alternative therapeutic approach to systemic administration of brain-derived GM1 to enhance GM1 levels in the brain via enzymatic conversion of polysialogangliosides into GM1 and to assess the neuroprotective potential of this approach. Methods We used sialidase from Vibrio cholerae (VCS) to convert GD1a, GD1b and GT1b gangliosides to GM1. VCS was infused by osmotic minipump into the dorsal third ventricle in mice over a 4-week period. After the first week of infusion, animals received MPTP injections (20 mg/kg, s.c., twice daily, 4 hours apart, for 5 consecutive days) and were euthanized 2 weeks after the last injection. Results VCS infusion resulted in the expected change in ganglioside expression with a significant increase in GM1 levels. VCS-treated animals showed significant sparing of striatal dopamine (DA) levels and substantia nigra DA neurons following MPTP administration, with the extent of sparing of DA neurons similar to that achieved with systemic GM1 administration. Conclusion The results suggest that enzymatic conversion of polysialogangliosides to GM1 may be a viable treatment strategy for increasing GM1 levels in the brain and exerting a neuroprotective effect on the damaged nigrostriatal DA system. PMID:26629687

  4. GM2 gangliosidosis in British Jacob sheep.

    PubMed

    Wessels, M E; Holmes, J P; Jeffrey, M; Jackson, M; Mackintosh, A; Kolodny, E H; Zeng, B J; Wang, C B; Scholes, S F E

    2014-01-01

    GM2 gangliosidosis (Tay-Sachs disease) was diagnosed in 6- to 8-month-old pedigree Jacob lambs from two unrelated flocks presenting clinically with progressive neurological dysfunction of 10 day's to 8 week's duration. Clinical signs included hindlimb ataxia and weakness, recumbency and proprioceptive defects. Histopathological examination of the nervous system identified extensive neuronal cytoplasmic accumulation of material that stained with periodic acid--Schiff and Luxol fast blue. Electron microscopy identified membranous cytoplasmic bodies within the nervous system. Serum biochemistry detected a marked decrease in hexosaminidase A activity in the one lamb tested, when compared with the concentration in age matched controls and genetic analysis identified a mutation in the sheep hexa allele G444R consistent with Tay-Sachs disease in Jacob sheep in North America. The identification of Tay-Sachs disease in British Jacob sheep supports previous evidence that the mutation in North American Jacob sheep originated from imported UK stock. PMID:24309906

  5. A photoreactive derivative of radiolabeled GM1 ganglioside: Preparation and use to establish the involvement of specific proteins in GM1 uptake by human fibroblasts in culture

    SciTech Connect

    Sonnino, S.; Chigorno, V.; Acquotti, D.; Pitto, M.; Kirschner, G.; Tettamanti, G. )

    1989-01-10

    A new procedure was used to synthesize a derivative of ganglioside GM1 containing a photoreactive nitrophenyl azide group at the end of the fatty acyl moiety, using deAc-deAcyl-GM1 obtained by deacetylation of the sialic acid and deacylation of the ceramide portion of GM1. This deAc-deAcyl-GM1 was first acylated at the long chain base amino group with 12-aminododecanoic acid, which has the amino group protected by a fluorenyl residue, and tritium labeled at the sialic acid amino group with ({sup 3}H)acetic anhydride of very high specific radioactivity. Cultured human fibroblasts were exposed to mixtures of radioactive photolabeled GM1 for different times and then illuminated and the radioactive protein patterns studied by SDS-PAGE. After 2 h of exposure, the photolabeled GM1 was stably associated to the cells and underwent almost no metabolic processing, behaving exactly as the underivatized natural GM1. Under these conditions very few proteins became radioactive. Thus, it is evident that the ganglioside binding to fibroblasts and insertion into the outer layer of the plasma membrane involve few individual proteins. When the incubation was prolonged to 24 h, photolabeled GM1 underwent extensive metabolic processing and gave origins to the corresponding ganglioside derivatives of GM2, GM3, and GD1a. Under these conditions many proteins became radioactive, a consequence of GM1 transfer from the surface to the interior or the cell and of the ready availability of interaction of GM1 and its metabolites.

  6. Pyrimethamine as a potential pharmacological chaperone for late-onset forms of GM2 gangliosidosis.

    PubMed

    Maegawa, Gustavo H B; Tropak, Michael; Buttner, Justin; Stockley, Tracy; Kok, Fernando; Clarke, Joe T R; Mahuran, Don J

    2007-03-23

    Late-onset GM2 gangliosidosis is composed of two related, autosomal recessive, neurodegenerative diseases, both resulting from deficiency of lysosomal, heterodimeric beta-hexosaminidase A (Hex A, alphabeta). Pharmacological chaperones (PC) are small molecules that can stabilize the conformation of a mutant protein, allowing it to pass the quality control system of the endoplasmic reticulum. To date all successful PCs have also been competitive inhibitors. Screening for Hex A inhibitors in a library of 1040 Food Drug Administration-approved compounds identified pyrimethamine (PYR (2,4-diamino 5-(4-chlorophenyl)-6-ethylpyrimidine)) as the most potent inhibitor. Cell lines from 10 late-onset Tay-Sachs (11 alpha-mutations, 2 novel) and 7 Sandhoff (9 beta-mutations, 4 novel) disease patients, were cultured with PYR at concentrations corresponding to therapeutic doses. Cells carrying the most common late-onset mutation, alphaG269S, showed significant increases in residual Hex A activity, as did all 7 of the beta-mutants tested. Cells responding to PC treatment included those carrying mutants resulting in reduced Hex heat stability and partial splice junction mutations of the inherently less stable alpha-subunit. PYR, which binds to the active site in domain II, was able to function as PC even to domain I beta-mutants. We concluded that PYR functions as a mutation-specific PC, variably enhancing residual lysosomal Hex A levels in late-onset GM2 gangliosidosis patient cells. PMID:17237499

  7. Ganglioside GM1 mimicry in Campylobacter strains from sporadic infections in the United States.

    PubMed

    Nachamkin, I; Ung, H; Moran, A P; Yoo, D; Prendergast, M M; Nicholson, M A; Sheikh, K; Ho, T; Asbury, A K; McKhann, G M; Griffin, J W

    1999-05-01

    To determine whether GM1-like epitopes in Campylobacter species are specific to O serotypes associated with Guillain-Barré syndrome (GBS) or whether they are frequent among random Campylobacter isolates causing enteritis, 275 random enteritis-associated isolates of Campylobacter jejuni were analyzed. To determine whether GM1-like epitopes in Campylobacter species are specific to O serotypes associated with Guillan-Barre syndrome (GBS) or whether they are frequent among random Campylobacter isolates causing enteritis, 275 enteritis-associated isolates, randomly collected in the United States, were analyzed using a cholera-toxin binding assay [corrected]. Overall, 26.2% of the isolates were positive for the GM1-like epitope. Of the 36 different O serotypes in the sample, 21 (58.3%) contained no strains positive for GM1, whereas in 6 serotypes (16.7%), >50% of isolates were positive for GM1. GBS-associated serotypes were more likely to contain strains positive for GM1 than were non-GBS-associated serotypes (37.8% vs. 15.1%, P=.0116). The results suggest that humans are frequently exposed to strains exhibiting GM1-like mimicry and, while certain serotypes may be more likely to possess GM1-like epitopes, the presence of GM1-like epitopes on Campylobacter strains does not itself trigger GBS. PMID:10191221

  8. Condensing and Fluidizing Effects of Ganglioside GM1 on Phospholipid Films

    PubMed Central

    Frey, Shelli L.; Chi, Eva Y.; Arratia, Cristóbal; Majewski, Jaroslaw; Kjaer, Kristian; Lee, Ka Yee C.

    2008-01-01

    Mixed monolayers of the ganglioside GM1 and the lipid dipalmitoylphosphatidlycholine (DPPC) at air-water and solid-air interfaces were investigated using various biophysical techniques to ascertain the location and phase behavior of the ganglioside molecules in a mixed membrane. The effects induced by GM1 on the mean molecular area of the binary mixtures and the phase behavior of DPPC were followed for GM1 concentrations ranging from 5 to 70 mol %. Surface pressure isotherms and fluorescence microscopy imaging of domain formation indicate that at low concentrations of GM1 (<25 mol %), the monolayer becomes continually more condensed than DPPC upon further addition of ganglioside. At higher GM1 concentrations (>25 mol %), the mixed monolayer becomes more expanded or fluid-like. After deposition onto a solid substrate, atomic force microscopy imaging of these lipid monolayers showed that GM1 and DPPC pack cooperatively in the condensed phase domain to form geometrically packed complexes that are more ordered than either individual component as evidenced by a more extended total height of the complex arising from a well-packed hydrocarbon tail region. Grazing incidence x-ray diffraction on the DPPC/GM1 binary mixture provides evidence that ordering can emerge when two otherwise fluid components are mixed together. The addition of GM1 to DPPC gives rise to a unit cell that differs from that of a pure DPPC monolayer. To determine the region of the GM1 molecule that interacts with the DPPC molecule and causes condensation and subsequent expansion of the monolayer, surface pressure isotherms were obtained with molecules modeling the backbone or headgroup portions of the GM1 molecule. The observed concentration-dependent condensing and fluidizing effects are specific to the rigid, sugar headgroup portion of the GM1 molecule. PMID:18192361

  9. A pilot study with monosialoganglioside GM1 on acute cerebral ischemia.

    PubMed

    Giraldi, C; Masi, M C; Manetti, M; Carabelli, E; Martini, A

    1990-06-01

    Reported here are the results of an open controlled study on the use of GM1 in cases of ischemic strokes in its acute phase. A statistically significant improvement was observed in cases treated with GM1 for neurological deficits (assessed by Mathew's rating scale, modified by Fritz-Werner) at 21, 60 and 120 days and for disability at 120 days. PMID:2206015

  10. Amyloid β-peptide insertion in liposomes containing GM1-cholesterol domains.

    PubMed

    Nicastro, Maria Carmela; Spigolon, Dario; Librizzi, Fabio; Moran, Oscar; Ortore, Maria Grazia; Bulone, Donatella; Biagio, Pier Luigi San; Carrotta, Rita

    2016-01-01

    Neuronal membrane damage is related to the early impairments appearing in Alzheimer's disease due to the interaction of the amyloid β-peptide (Aβ) with the phospholipid bilayer. In particular, the ganglioside GM1, present with cholesterol in lipid rafts, seems to be able to initiate Aβ aggregation on membrane. We studied the thermodynamic and structural effects of the presence of GM1 on the interaction between Aβ and liposomes, a good membrane model system. Isothermal Titration Calorimetry highlighted the importance of the presence of GM1 in recruiting monomeric Aβ toward the lipid bilayer. Light and Small Angle X-ray Scattering revealed a different pattern for GM1 containing liposomes, both before and after interaction with Aβ. The results suggest that the interaction with GM1 brings to insertion of Aβ in the bilayer, producing a structural perturbation down to the internal layers of the liposome, as demonstrated by the obtained electron density profiles. PMID:26259785

  11. GM1 erythroimmunoassay for detection and titration of Escherichia coli heat-labile enterotoxin.

    PubMed

    Germani, Y; Bégaud, E; Guesdon, J L; Moreau, J P

    1986-11-01

    A GM1 ganglioside erythroimmunoassay for the detection of heat-labile Escherichia coli enterotoxin (LT) was developed for use in poorly equipped laboratories in developing countries. This assay is based on the immunological similarity between Vibrio cholerae toxin and LT and uses cholera toxin antiserum and sheep anti-rabbit immunoglobulin covalently coupled to sheep erythrocytes as conjugate. This assay has the following advantages over other currently available techniques: the reagents it uses are stable, in particular, tanned and sensitized sheep erythrocytes; GM1 ganglioside is commercially available; erythro-adsorption can be read with the naked eye; the test can be completed in 1 day; and as little as 4 ng of V. cholerae toxin or LT per ml can be detected accurately. The GM1 ganglioside erythroimmunoassay showed good quantitative and qualitative correlation with the Vero cell assay and the conventional GM1 enzyme-linked immunosorbent assay. The GM1 ganglioside erythroimmunoassay was somewhat less sensitive than the GM1 enzyme-linked immunosorbent assay but more sensitive than the Vero cell assay. Results obtained for 12 LT-positive and 138 LT-negative E. coli strains correlated with results obtained with GM1 enzyme-linked immunosorbent and Vero cell assays. PMID:3533985

  12. Changes in GM1 ganglioside content and localization in cholestatic rat liver.

    PubMed

    Jirkovská, Marie; Majer, Filip; Smídová, Jaroslava; Stríteský, Jan; Shaik, Gouse Mohiddin; Dráber, Petr; Vítek, Libor; Marecek, Zdenek; Smíd, Frantisek

    2007-07-01

    (Glyco)sphingolipids (GSL) are believed to protect the cell against harmful environmental factors by increasing the rigidity of plasma membrane. Marked decrease of membrane fluidity in cholestatic hepatocytes was described but the role of GSL therein has not been investigated so far. In this study, localization in hepatocytes of a representative of GSL, the GM1 ganglioside, was compared between of rats with cholestasis induced by 17alpha-ethinylestradiol (EE) and vehicle propanediol treated or untreated animals. GM1 was monitored by histochemical reaction employing cholera toxin B-subunit. Our findings in normal rat liver tissue showed that GM1 was localized in sinusoidal and canalicular hepatocyte membranes in both peripheral and intermediate zones of the hepatic lobules, and was nearly absent in central zones. On the contrary, in EE-treated animals GM1 was also expressed in central lobular zones. Moreover, detailed densitometry analysis at high magnification showed greater difference of GM1 expression between sinusoidal surface areas and areas of adjacent cytoplasm, caused as well by increased sinusoidal staining in central lobular zone as by decreased staining in cytoplasm in peripheral zone. These differences correlated with serum bile acids as documented by linear regression analyses. Both GM1 content and mRNA corresponding to GM1-synthase remained unchanged in livers; the enhanced expression of GM1 at sinusoidal membrane thus seems to be due to re-distribution of cellular GM1 at limited biosynthesis and could be responsible for protection of hepatocytes against harmful effects of bile acids accumulated during cholestasis. PMID:17333356

  13. Recovery from Experimental Parkinsonism in Primates with GM1 Ganglioside Treatment

    NASA Astrophysics Data System (ADS)

    Schneider, J. S.; Pope, Anne; Simpson, Kimberly; Taggart, James; Smith, M. G.; Distefano, L.

    1992-05-01

    A parkinsonian syndrome can be produced in nonhuman primates by administration of the neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). Parkinsonian-like symptoms induced acutely by MPTP were ameliorated after treatment with GM1 ganglioside, a substance shown to have neurotrophic effects on the damaged dopamine system in rodents. Treatment with GM1 ganglioside also increased striatal dopamine and metabolite levels and enhanced the dopaminergic innervation of the striatum as demonstrated by tyrosine hydroxylase immunohistochemistry. These results suggest that GM1 ganglioside may hold promise as a therapeutic agent for the treatment of Parkinson's disease.

  14. Neuropathology of chronic GM2 gangliosidosis due to hexosaminidase A deficiency.

    PubMed

    Kornfeld, M

    2008-01-01

    Autopsy studies of late-onset GM2 gangliosidosis are sparse and only one adult case is on record. The case of partial Hex A deficiency presented here started in childhood as spinal muscular atrophy which progressed slowly over 4 decades. Cognitive function remained intact throughout the entire course, but during the last few years of life allodynia supervened. The patient died at 44 years of age. In good correlation with clinical observations the autopsy findings showed the most severe accumulation of lipid and consequent regressive change in the anterior horns of the spinal cord. Extensive but less severe storage was found in other spinal cord neurons, brain stem and selected basal ganglia. Cerebral cortex was virtually spared by storage but was the site of excessive formation of lipofuscin which was also present in many other neurons in the CNS. Marked storage and ganglionic loss was also found in the dorsal root ganglia, and the fasciculus gracilis was severely depleted of myelinated fibers. Electron microscopy showed accumulated gangliosides almost exclusively in the form of single and coalescing zebra bodies. In conclusion, the pathology in this case of chronic GM2 gangliosidosis, though in part conforming with previous observations, differed in several aspects. First, the cerebral cortex was--with only a few exceptions--free of ganglioside storage. Also spared was the cerebellum. In addition, homogeneous accumulation of zebra bodies contrasted with heterogeneity of neuronal inclusions found in other chronic cases. Finally, the involvement of sensory neurons was prominent and potentially related to allodynia. Molecular study of HEXA gene in this patient showed an TATC1278/? genotype. PMID:18808061

  15. MECHANISMS UNDERLYING THE MICRON-SCALE SEGREGATION OF STEROLS AND GM1 IN LIVE MAMMALIAN SPERM

    PubMed Central

    Selvaraj, Vimal; Asano, Atsushi; Buttke, Danielle E.; Sengupta, Prabuddha; Weiss, Robert S.; Travis, Alexander J.

    2009-01-01

    We demonstrate for the first time that a stable, micron-scale segregation of focal enrichments of sterols exists at physiological temperature in the plasma membrane of live murine and human sperm. These enrichments of sterols represent microheterogeneities within this membrane domain overlying the acrosome. Previously, we showed that cholera toxin subunit B (CTB), which binds the glycosphingolipid, GM1, localizes to this same domain in live sperm. Interestingly, the GM1 undergoes an unexplained redistribution upon cell death. We now demonstrate that GM1 is also enriched in the acrosome, an exocytotic vesicle. Transfer of lipids between this and the plasma membrane occurs at cell death, increasing GM1 in the plasma membrane without apparent release of acrosomal contents. This finding provides corroborative support for an emerging model of regulated exocytosis in which membrane communications might occur without triggering the “acrosome reaction.” Comparison of the dynamics of CTB-bound endogenous GM1 and exogenous BODIPY-GM1 in live murine sperm demonstrate that the sub-acrosomal ring functions as a specialized diffusion barrier segregating specific lipids within the sperm head plasma membrane. Our data show significant differences between endogenous lipids and exogenous lipid probes in terms of lateral diffusion. Based on these studies, we propose a hierarchical model to explain the segregation of this sterol- and GM1-enriched domain in live sperm, which is positioned to regulate sperm fertilization competence and mediate interactions with the oocyte. Moreover, our data suggest potential origins of sub-types of membrane raft microdomains enriched in sterols and/or GM1 that can be separated biochemically. PMID:19012288

  16. Galleria mellonella native and analogue peptides Gm1 and ΔGm1. II) anti-bacterial and anti-endotoxic effects.

    PubMed

    Correa, Wilmar; Manrique-Moreno, Marcela; Behrends, Jochen; Patiño, Edwin; Marella, Chakravarthy; Peláez-Jaramillo, Carlos; Garidel, Patrick; Gutsmann, Thomas; Brandenburg, Klaus; Heinbockel, Lena

    2014-10-01

    Antimicrobial peptides (AMPs) are important components of the innate immune system of animals, plants, fungi and bacteria and are recently under discussion as promising alternatives to conventional antibiotics. We have investigated two cecropin-like synthetic peptides, Gm1, which corresponds to the natural overall uncharged Galleria mellonella native peptide and ΔGm1, a modified overall positively charged Gm1 variant. We have analysed these peptides for their potential to inhibit the endotoxin-induced secretion of tumour necrosis factor-α (TNF-α) from human mononuclear cells. Furthermore, in a conventional microbiological assay, the ability of these peptides to inhibit the growth of the rough mutant bacteria Salmonella enterica Minnesota R60 and the polymyxin B-resistant Proteus mirabilis R45 was investigated and atomic force microscopy (AFM) measurements were performed to characterize the morphology of the bacteria treated by the two peptides. We have also studied their cytotoxic properties in a haemolysis assay to clarify potential toxic effects. Our data revealed for both peptides minor anti-inflammatory (anti-endotoxin) activity, but demonstrated antimicrobial activity with differences depending on the endotoxin composition of the respective bacteria. In accordance with the antimicrobial assay, AFM data revealed a stronger morphology change of the R45 bacteria than for the R60. Furthermore, Gm1 had a stronger effect on the bacteria than ΔGm1, leading to a different morphology regarding indentations and coalescing of bacterial structures. The findings verify the biophysical measurements with the peptides on model systems. Both peptides lack any haemolytic activity up to an amount of 100μg/ml, making them suitable as new anti-infective agents. PMID:25016054

  17. Galleria mellonella native and analogue peptides Gm1 and ΔGm1. I) biophysical characterization of the interaction mechanisms with bacterial model membranes.

    PubMed

    Correa, Wilmar; Manrique-Moreno, Marcela; Patiño, Edwin; Peláez-Jaramillo, Carlos; Kaconis, Yani; Gutsmann, Thomas; Garidel, Patrick; Heinbockel, Lena; Brandenburg, Klaus

    2014-10-01

    Natural occurring antimicrobial peptides (AMPs) are important components of the innate immune system of animals and plants. They are considered to be promising alternatives to conventional antibiotics. Here we present a comparative study of two synthetic peptides: Gm1, corresponding to the natural overall uncharged peptide from Galleria mellonella (Gm) and ΔGm1, a modified overall positively charged Gm1 variant. We have studied the interaction of the peptides with lipid membranes composed of different kinds of lipopolysaccharides (LPS) and dimyristoylphosphatidylglycerol (DMPG), in some cases also dimyristoylphosphatidylethanolamine (DMPE) as representative lipid components of Gram-negative bacterial membranes, by applying Fourier-transform infrared spectroscopy (FTIR), Förster resonance energy transfer spectroscopy (FRET), differential scanning calorimetry (DSC) and isothermal titration calorimetry (ITC). Gm1 generates a destabilizing effect on the gel to liquid crystalline phase transition of the acyl chains of the lipids, as deduced from a decrease in the phase transition temperature and enthalpy, suggesting a fluidization, whereas ΔGm1 led to the opposite behavior. Further, FTIR analysis of the functional groups of the lipids participating in the interaction with the peptides indicated a shift in the band position and intensity of the asymmetric PO2(-) stretching vibration originating from the lipid phosphate groups, a consequence of the sterical changes in the head group region. Interestingly, FRET spectroscopy showed a similar intercalation of both peptides into the DMPG and LPS, but much less into the DMPE membrane systems. These results are discussed in the light of a possible use of the peptides as antimicrobial and anti-endotoxin drugs. PMID:25017800

  18. Complement Factor H and Simian Virus 40 bind the GM1 ganglioside in distinct conformations.

    PubMed

    Blaum, Bärbel S; Frank, Martin; Walker, Ross C; Neu, Ursula; Stehle, Thilo

    2016-05-01

    Mammalian cell surfaces are decorated with a variety of glycan chains that orchestrate development and defense and are exploited by pathogens for cellular attachment and entry. While glycosidic linkages are, in principle, flexible, the conformational space that a given glycan can sample is subject to spatial and electrostatic restrictions imposed by its overall chemical structure. Here, we show how the glycan moiety of the GM1 ganglioside, a branched, monosialylated pentasaccharide that serves as a ligand for various proteins, undergoes differential conformational selection in its interactions with different lectins. Using STD NMR and X-ray crystallography, we found that the innate immune regulator complement Factor H (FH) binds a previously not reported GM1 conformation that is not compatible with the GM1-binding sites of other structurally characterized GM1-binding lectins such as the Simian Virus 40 (SV40) capsid. Molecular dynamics simulations of the free glycan in explicit solvent on the 10 μs timescale reveal that the FH-bound conformation nevertheless corresponds to a minimum in the Gibbs free energy plot. In contrast to the GM1 conformation recognized by SV40, the FH-bound GM1 conformation is associated with poor NOE restraints, explaining how it escaped(1)H-(1)H NOE-restrained modeling in the past and highlighting the necessity for ensemble representations of glycan structures. PMID:26715202

  19. Ganglioside GM1 Contributes to the State of Insulin Resistance in Senescent Human Arterial Endothelial Cells.

    PubMed

    Sasaki, Norihiko; Itakura, Yoko; Toyoda, Masashi

    2015-10-16

    Vascular endothelial cells (ECs) play central roles in physiologically important functions of blood vessels and contribute to the maintenance of vascular integrity. Therefore, it is considered that the impairment of EC functions leads to the development of vascular diseases. However, the molecular mechanisms of the EC dysfunctions that accompany senescence and aging have not yet been clarified. The carbohydrate antigens carried by glycoconjugates (e.g. glycoproteins, glycosphingolipids, and proteoglycans) mainly present on the cell surface serve not only as marker molecules but also as functional molecules. In this study, we have investigated the abundance and functional roles of glycosphingolipids in human ECs during senescence and aging. Among glycosphingolipids, ganglioside GM1 was highly expressed in abundance on the surface of replicatively and prematurely senescent ECs and also of ECs derived from an elderly subject. Insulin signaling, which regulates important functions of ECs, is impaired in senescent and aged ECs. Actually, by down-regulating GM1 on senescent ECs and overloading exogenous GM1 onto non-senescent ECs, we showed that an increased abundance of GM1 functionally contributes to the impairment of insulin signaling in ECs. Taken together, these findings provide the first evidence that GM1 increases in abundance on the cell surface of ECs under the conditions of cellular senescence and aging and causes insulin resistance in ECs. GM1 may be an attractive target for the detection, prevention, and therapy of insulin resistance and related vascular diseases, particularly in older people. PMID:26338710

  20. Ganglioside GM1 Contributes to the State of Insulin Resistance in Senescent Human Arterial Endothelial Cells*

    PubMed Central

    Sasaki, Norihiko; Itakura, Yoko; Toyoda, Masashi

    2015-01-01

    Vascular endothelial cells (ECs) play central roles in physiologically important functions of blood vessels and contribute to the maintenance of vascular integrity. Therefore, it is considered that the impairment of EC functions leads to the development of vascular diseases. However, the molecular mechanisms of the EC dysfunctions that accompany senescence and aging have not yet been clarified. The carbohydrate antigens carried by glycoconjugates (e.g. glycoproteins, glycosphingolipids, and proteoglycans) mainly present on the cell surface serve not only as marker molecules but also as functional molecules. In this study, we have investigated the abundance and functional roles of glycosphingolipids in human ECs during senescence and aging. Among glycosphingolipids, ganglioside GM1 was highly expressed in abundance on the surface of replicatively and prematurely senescent ECs and also of ECs derived from an elderly subject. Insulin signaling, which regulates important functions of ECs, is impaired in senescent and aged ECs. Actually, by down-regulating GM1 on senescent ECs and overloading exogenous GM1 onto non-senescent ECs, we showed that an increased abundance of GM1 functionally contributes to the impairment of insulin signaling in ECs. Taken together, these findings provide the first evidence that GM1 increases in abundance on the cell surface of ECs under the conditions of cellular senescence and aging and causes insulin resistance in ECs. GM1 may be an attractive target for the detection, prevention, and therapy of insulin resistance and related vascular diseases, particularly in older people. PMID:26338710

  1. Efficient biotransformation of polysialogangliosides for preparation of GM1 by Cellulosimicrobium sp. 21.

    PubMed

    Zheng, Yan; Ji, Li; Leng, Jiayi; Yuan, Ye; Chen, Honglei; Gou, Dongxia; Gao, Yufei; Zhou, Yifa

    2014-01-01

    A new ganglioside transformed strain isolated from soil was identified as Cellulosimicrobium sp. 21. It produced a sialidase which transformed polysialo-gangliosides GD1 and GT1 into a monosialoterahexosylganglioside, i.e., ganglioside GM1. The sialidase had both NeuAc-α-2,3- and NeuAc-α-2,8-sialidase activity without producing asiolo-GM1. The optimum conditions were evaluated and it was found that the transformation was optimally performed at 30 °C and pH 7.0. The substrate should be added at the beginning of the reaction and the concentration of substrate was 3% (w/v). Under these optimum conditions, Cellulosimicrobium sp. 21 converted GD1 and GT1 into GM1 in inorganic medium in a 5 L bioreactor with the recovery rate of 69.3%. The product contained 50.3% GM1 and was purified on silica to give the product with 95% of GM1 with a recovery rate of 30.5%. Therefore, Cellulosimicrobium sp. 21 has potential to be applied in the production of GM1 in the pharmaceutical industry. PMID:25299818

  2. Pathology of GM2 gangliosidosis in Jacob sheep.

    PubMed

    Porter, B F; Lewis, B C; Edwards, J F; Alroy, J; Zeng, B J; Torres, P A; Bretzlaff, K N; Kolodny, E H

    2011-07-01

    The G(M2) gangliosidoses are a group of lysosomal storage diseases caused by defects in the genes coding for the enzyme hexosaminidase or the G(M2) activator protein. Four Jacob sheep from the same farm were examined over a 3-year period for a progressive neurologic disease. Two lambs were 6-month-old intact males and 2 were 8-month-old females. Clinical findings included ataxia in all 4 limbs, proprioceptive deficits, and cortical blindness. At necropsy, the nervous system appeared grossly normal. Histologically, most neurons within the brain, spinal cord, and peripheral ganglia were enlarged, and the cytoplasm was distended by foamy to granular material that stained positively with Luxol fast blue and Sudan black B stains. Other neuropathologic findings included widespread astrocytosis, microgliosis, and scattered spheroids. Electron microscopy revealed membranous cytoplasmic bodies within the cytoplasm of neurons. Biochemical and molecular genetic studies confirmed the diagnosis of G(M2) gangliosidosis. This form of G(M2) gangliosidosis in Jacob sheep is very similar to human Tay-Sachs disease and is potentially a useful animal model. PMID:21123862

  3. Development of an Immunoassay for Rapid Detection of Ganglioside GM1 Mimicry in Campylobacter jejuni Strains

    PubMed Central

    Prendergast, Martina M.; Kosunen, Timo U.; Moran, Anthony P.

    2001-01-01

    Mimicry of peripheral nerve gangliosides by Campylobacter jejuni lipopolysaccharides (LPSs) has been proposed to induce cross-reacting antiganglioside antibodies in Guillain-Barré syndrome (GBS). Because current methods for LPS characterization are labor-intensive and inhibit the screening of large numbers of strains, a rapid GM1 epitope screening assay was developed. Biomass from two agar plates of confluent growth yielded sufficient LPS using a novel phenol-water and ether extraction procedure. Extracts of LPS were reacted with cholera toxin (GM1 ligand), peanut agglutinin (Galβ1→3GalNAc ligand), and anti-GM1 antibodies. After the assay was validated, 12 of 59 (20%) C. jejuni serostrains, including four serotypes that have not previously been associated with GBS, reacted with two or more anti-GM1 ganglioside reagents. Subsequently, LPS extracts from 5 of 7 (71%) C. jejuni isolates and 2 of 3 (67%) C. jejuni culture collection strains bore GM1 structures. Overall, the assay system was reliable, efficient, and reproducible and may be adapted for large-scale epidemiological studies. PMID:11283076

  4. Pili and lipopolysaccharide of Pseudomonas aeruginosa bind to the glycolipid asialo GM1.

    PubMed Central

    Gupta, S K; Berk, R S; Masinick, S; Hazlett, L D

    1994-01-01

    This study investigated which adhesins of Pseudomonas aeruginosa interact with the glycolipid asialo GM1, using solid-phase binding and thin-layer chromatography assays. Radioiodinated pili and flagella contaminated with lipopolysaccharide (LPS) bound to the glycolipid. When LPS was reduced to acceptable levels in pilus and flagellum samples, only pili specifically bound to the glycolipid. Commercial, radiolabeled LPS as well as whole bacteria of strain ATCC 19660 also bound to asialo GM1. Binding was specific, competitive, and saturable. Organ cultures of whole mouse eyes and scanning electron microscopy techniques were used also, and strain ATCC 19660 was inhibited from corneal binding by exogenous pili or commercial LPS and inhibition was concentration dependent for both. Binding of radiolabeled strain ATCC 19660 bacteria to neutral lipids extracted from bovine corneal epithelial tissue showed that the bacteria bound to a glycolipid which migrated at a position similar to that of an asialo GM1 standard and that the glycolipid stained positively with an antibody specific for asialo GM1. The data provide evidence that pili (reduced LPS) and LPS of P. aeruginosa bind to asialo GM1 glycolipid and that the glycolipid is not restricted to the mouse cornea. Images PMID:7927723

  5. Bis(monoacylglycero)phosphate and ganglioside GM1 spontaneously form small homogeneous vesicles at specific concentrations

    SciTech Connect

    Chebukati, Janetricks N.; Goff, Philip C.; Frederick, Thomas E.; Fanucci, Gail E.

    2010-04-09

    The morphology and size of hydrated lipid dispersions of bis(monoacylglycero)phosphate (BMP) mixed with varying mole percentages of the ganglioside GM1 were investigated by dynamic light scattering (DLS) and transmission electron microscopy (TEM). Electron paramagnetic resonance (EPR) spectroscopy of these same mixtures, doped at 0.5 mol% with doxyl labeled lipids, was used to investigate acyl-chain packing. Results show that for 20-30% GM1, hydrated BMP:GM1 mixtures spontaneously form small spherical vesicles with diameters {approx}100 nm and a narrow size distribution profile. For other concentrations of GM1, hydrated dispersions with BMP have non-spherical shapes and heterogeneous size profiles, with average vesicle diameters >400 nm. All samples were prepared at pH 5.5 to mimic the lumen acidity of the late endosome where BMP is an essential component of intraendosomal vesicle budding, lipid sorting and trafficking. These findings indicate that GM1 and BMP under a limited concentration range spontaneously form small vesicles of homogeneous size in an energy independent manner without the need of protein templating. Because BMP is essential for intraendosomal vesicle formation, these results imply that lipid-lipid interactions may play a critical role in the endosomal process of lipid sorting and trafficking.

  6. The multi-tasked life of GM1 ganglioside, a true factotum of nature.

    PubMed

    Ledeen, Robert W; Wu, Gusheng

    2015-07-01

    GM1 ganglioside occurs widely in vertebrate tissues, where it exhibits many essential functions, both in the plasma membrane and intracellular loci. Its essentiality is revealed in the dire consequences resulting from genetic deletion. This derives from its key roles in several signalosome systems, characteristically located in membrane rafts, where it associates with specific proteins that have glycolipid-binding domains. Thus, GM1 interacts with proteins that modulate mechanisms such as ion transport, neuronal differentiation, G protein-coupled receptors (GPCRs), immune system reactivities, and neuroprotective signaling. The latter occurs through intimate association with neurotrophin receptors, which has relevance to the etiopathogenesis of neurodegenerative diseases and potential therapies. Here, we review the current state of knowledge of these GM1-associated mechanisms. PMID:26024958

  7. Protection against Experimental Stroke by Ganglioside GM1 Is Associated with the Inhibition of Autophagy

    PubMed Central

    Li, Li; Tian, Jinghua; Long, Mitchell King-Wei; Chen, Yong; Lu, Jianfei; Zhou, Changman; Wang, Tianlong

    2016-01-01

    Ganglioside GM1, which is particularly abundant in the central nervous system (CNS), is closely associated with the protection against several CNS disorders. However, controversial findings have been reported on the role of GM1 following ischemic stroke. In the present study, using a rat middle cerebral artery occlusion (MCAO) model, we investigated whether GM1 can protect against ischemic brain injury and whether it targets the autophagy pathway. GM1 was delivered to Sprague-Dawley male rats at 3 doses (25 mg/kg, 50 mg/kg, 100 mg/kg) by intraperitoneal injection soon after reperfusion and then once daily for 2 days. The same volume of saline was given as a control. Tat–Beclin-1, a specific autophagy inducer, was administered by intraperitoneal injection at 24 and 48 hours post-MCAO. Infarction volume, mortality and neurological function were assessed at 72 hours after ischemic insult. Immunofluorescence and Western blotting were performed to determine the expression of autophagy-related proteins P62, LC3 and Beclin-1 in the penumbra area. No significant changes in mortality and physiological variables (heart rate, blood glucose levels and arterial blood gases) were observed between the different groups. However, MCAO resulted in enhanced conversion of LC3-I into LC3-II, P62 degradation, high levels of Beclin-1, a large area infarction (26.3±3.6%) and serious neurobehavioral deficits. GM1 (50 mg/kg) treatment significantly reduced the autophagy activation, neurobehavioral dysfunctions, and infarction volume (from 26.3% to 19.5%) without causing significant adverse side effects. However, this biological function could be abolished by Tat–Beclin-1. In conclusion: GM1 demonstrated safe and robust neuroprotective effects that are associated with the inhibition of autophagy following experimental stroke. PMID:26751695

  8. Ordering Transitions in Micrometer-Thick Films of Nematic Liquid Crystals Driven by Self-Assembly of Ganglioside GM1

    PubMed Central

    Lin, I-Hsin; Meli, Maria-Victoria; Abbott, Nicholas L.

    2009-01-01

    We report an investigation of the self-assembly of the monosialoganglioside (GM1) at interfaces formed between aqueous solutions of 10 µM GM1 (at 25°C) and micrometer-thick films of the nematic liquid crystal (LC) 4’-pentyl-4-cyanobiphenyl (5CB). We observe the process of spontaneous transfer of GM1 onto the interfaces to be accompanied by continuous ordering transitions within the micrometer-thick films of the LC. At saturation coverage, the GM1 orders the LC in an orientation that is perpendicular to the interface, an orientation that is similar to that caused by phospholipids such as dilaurylphosphatidylcholine (DLPC). This result suggests an interaction between the LC and GM1 that is dominated by the hydrophobic tails of the GM1. Relative to DLPC, however, we observe the dynamics of the LC ordering transition driven by GM1 to be slow (2 hours for DLPC versus 100 hours for GM1). To provide insight into the origins of the slow dynamics of the GM1-induced ordering transition in the LC, we performed two additional measurements. First, we quantified the time-dependent adsorption of GM1 at the LC interface by using fluorescently-labeled GM1. Second, we used the Langmuir-Schaefer method to transfer preorganized monolayers of GM1 from an air-water interface to the aqueous-LC interface. Results obtained from these two experiments are consistent with a physical picture in which the final stages of spontaneous adsorption/ordering of GM1 at the aqueous-LC interface dictate the dynamics of the LC ordering transition. This rate limiting process underlying the ordering transition was substantially accelerated by heating the system above the phase transition temperature of GM1 (26 °C), suggesting that the phase state of the GM1 micellar aggregates in bulk solution strongly influences the kinetics of the final stages of ordering/adsorption of GM1 at the LC interface. Overall, these results and others presented in this manuscript reveal that it is possible to decorated interfaces of a nematic LC with GM1, and that the assembly of GM1 at these interfaces impacts the dynamic and equilibrium ordering of the LC. PMID:19428021

  9. GM1 Ganglioside Treatment Facilitates Behavioral Recovery from Bilateral Brain Damage

    NASA Astrophysics Data System (ADS)

    Sabel, Bernhard A.; Slavin, Mary D.; Stein, Donald G.

    1984-07-01

    Adult rats with bilateral lesions of the caudate nucleus were treated with GM1 ganglioside. Although animals injected with a control solution were severely impaired in their ability to learn a complex spatial task, those treated with ganglioside were able to learn spatial reversals.

  10. Co-localization of the Ganglioside GM1 and Cholesterol Detected by Secondary Ion Mass Spectrometry

    PubMed Central

    Lozano, Mónica M.; Liu, Zhao; Sunnick, Eva; Janshoff, Andreas; Kumar, Krishna; Boxer, Steven G.

    2013-01-01

    The characterization of the lateral organization of components in biological membranes and the evolution of this arrangement in response to external triggers remains a major challenge. The concept of lipid rafts is widely invoked, however, direct evidence of the existence of these ephemeral entities remains elusive. We report here the use of Secondary Ion Mass Spectrometry (SIMS) to image the cholesterol-dependent cohesive phase separation of the ganglioside GM1 into nano and micro-scale assemblies in a canonical lipid raft composition of lipids. This assembly of domains was interrogated in a model membrane system composed of palmitoyl sphingomyelin (PSM), cholesterol, and an unsaturated lipid (dioleoylphosphatidylcholine, DOPC). Orthogonal isotopic labeling of every lipid bilayer component and monofluorination of GM1 allowed generation of molecule specific images using a NanoSIMS. Simultaneous detection of six different ion species in SIMS, including secondary electrons, was used to generate ion ratio images whose signal intensity values could be correlated to composition through the use of calibration curves from standard samples. Images of this system provide the first direct, molecule specific, visual evidence for the co-localization of cholesterol and GM1 in supported lipid bilayers and further indicate the presence of three compositionally distinct phases: (1) the interdomain region; (2) micrometer-scale domains (d>3 μm); and, (3) nanometer-scale domains (d=100 nm − 1 μm) localized within the micrometer-scale domains and the interdomain region. PSM-rich, nanometer-scale domains prefer to partition within the more ordered, cholesterol-rich/DOPC-poor/GM1-rich micrometer-scale phase, while GM1-rich, nanometer-scale domains prefer to partition within the surrounding, disordered, cholesterol-poor/PSM-rich/DOPC-rich interdomain phase. PMID:23514537

  11. Colocalization of the ganglioside G(M1) and cholesterol detected by secondary ion mass spectrometry.

    PubMed

    Lozano, Mónica M; Liu, Zhao; Sunnick, Eva; Janshoff, Andreas; Kumar, Krishna; Boxer, Steven G

    2013-04-17

    The characterization of the lateral organization of components in biological membranes and the evolution of this arrangement in response to external triggers remain a major challenge. The concept of lipid rafts is widely invoked; however, direct evidence of the existence of these ephemeral entities remains elusive. We report here the use of secondary ion mass spectrometry (SIMS) to image the cholesterol-dependent cohesive phase separation of the ganglioside GM1 into nano- and microscale assemblies in a canonical lipid raft composition of lipids. This assembly of domains was interrogated in a model membrane system composed of palmitoyl sphingomyelin (PSM), cholesterol, and an unsaturated lipid (dioleoylphosphatidylcholine, DOPC). Orthogonal isotopic labeling of every lipid bilayer component and monofluorination of GM1 allowed generation of molecule specific images using a NanoSIMS. Simultaneous detection of six different ion species in SIMS, including secondary electrons, was used to generate ion ratio images whose signal intensity values could be correlated to composition through the use of calibration curves from standard samples. Images of this system provide the first direct, molecule specific, visual evidence for the colocalization of cholesterol and GM1 in supported lipid bilayers and further indicate the presence of three compositionally distinct phases: (1) the interdomain region; (2) micrometer-scale domains (d > 3 μm); (3) nanometer-scale domains (d = 100 nm to 1 μm) localized within the micrometer-scale domains and the interdomain region. PSM-rich, nanometer-scale domains prefer to partition within the more ordered, cholesterol-rich/DOPC-poor/GM1-rich micrometer-scale phase, while GM1-rich, nanometer-scale domains prefer to partition within the surrounding, disordered, cholesterol-poor/PSM-rich/DOPC-rich interdomain phase. PMID:23514537

  12. Fucosyl-GM1a, an endoglycoceramidase-resistant ganglioside of porcine brain.

    PubMed

    Xu, Xu; Monjusho, Hatsumi; Inagaki, Masanori; Hama, Yoichiro; Yamaguchi, Kuniko; Sakaguchi, Keishi; Iwamori, Masao; Okino, Nozomu; Ito, Makoto

    2007-01-01

    The use of bovine brain has been prohibited in many countries because of the world-wide prevalence of mad cow disease, and thus porcine brain is expected to be a new source for the preparation of gangliosides. Here, we report the presence of a ganglioside in porcine brain which is strongly resistant to hydrolysis by endoglycoceramidase, an enzyme capable of cleaving the glycosidic linkage between oligosaccharides and ceramides of various glycosphingolipids. Five major gangliosides (designated PBG-1, 2, 3, 4, 5) were extracted from porcine brain by Folch's partition, followed by mild alkaline hydrolysis and PBA column chromatography. We found that PBG-2, but not the others, was strongly resistant to hydrolysis by the enzyme. After the purification of PBG-2 with Q-Sepharose, Silica gel 60 and Prosep-PB chromatographies, the structure of PBG-2 was determined by GC, GC-MS, FAB-MS and NMR spectroscopy as Fucalpha1-2Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-4Glcbeta1-1'Cer (fucosyl-GM1a). The ceramide was mainly composed of C18:0 and C20:0 fatty acids and d18:1 and d20:1 sphingoid bases. The apparent kcat/Km for fucosyl-GM1a was found to be 30 times lower than that for GM1a, indicating that terminal fucosylation makes GM1a resistant to hydrolysis by the enzyme. This report indicates the usefulness of endoglycoceramidase to prepare fucosyl-GM1a from porcine brain. PMID:17167042

  13. Alpha-locus hexosaminidase genetic compound with juvenile gangliosidosis phenotype: clinical, genetic, and biochemical studies.

    PubMed Central

    Johnson, W G; Cohen, C S; Miranda, A F; Waran, S P; Chutorian, A M

    1980-01-01

    A 3-year-old boy developed progressive neurological deterioration in his third year, characterized by dementia, ataxia, myoclonic jerks, and bilateral macular cherry-red spots. Hexosaminidase A (HEX A) was partially decreased in the patient's serum, leukocytes, and cultured skin fibroblasts. Hexosaminidase was studied in serum and leukocytes from family members. Four members of the paternal branch appeared to be carriers of classical infantile Tay-Sachs allele, HEX alpha 2, probably receiving the gene from one great-grandparent of Ashkenazi origin. In the maternal branch, no one was a carrier of classical infantile Tay-Sachs disease, but five individuals were carriers of a milder alpha-locus defect. The patient, therefore, was a genetic compound of two different alpha-locus hexosaminidase mutations. At least 21 families with late-infantile or juvenile GM2 gangliosidosis have been reported, 18 of them with alpha-locus mutations, and three with beta-locus mutations. Genetic compounds of hexosaminidase have been reported in at least seven families, five with alpha-locus mutations and two with beta-locus mutations. The compound had the phenotype of infantile Tay-Sachs disease in one family, infantile Sandhoff disease in another, and the normal phenotype in the rest. PMID:6772023

  14. Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein.

    PubMed Central

    Inui, K; Grebner, E E; Jackson, L G; Wenger, D A

    1983-01-01

    Two sibling from a consanguineous Puerto Rican marriage were found to have a juvenile-onset type of lipidosis first noted at age 2 1/2 by expressing difficulties with motor function and developmental delay. They continued to deteriorate, showing muscle atrophy, spasticity, and loss of speech, and death occurred at ages 7 and 8. Examination of the brains from these patients revealed that the concentration of GM2 ganglioside was about 56% of the total gangliosides. Hexosaminidase and percent hexosaminidase A (HEX A) and other lysosomal enzymes were normal in cultured skin fibroblasts, liver, and brain. The concentration of the activator protein required for the enzymatic hydrolysis of GM2 ganglioside was in high normal levels in the brain of the patient available. However, the HEX A from the patient's brain and liver as well as from skin fibroblast lysates could not be activated to hydrolyze GM2 ganglioside by the activator protein from a control or himself. The HEX A from a control could be activated by the activator protein from controls or this patient. These patients appear to have a defect in HEX A, which does not affect it heat stability, electrophoretic migration, and activity toward fluorogenic substrates, but may affect the binding of the activator protein required for GM2 ganglioside hydrolysis. We propose to call these patients the AMB variant of GM2 gangliosidosis to denote the mutation in HEX A but with normal levels of HEX A and B with synthetic substrates. This is to distinguish these patients from those missing the activator protein and normal HEX A and B levels. Images Fig. 1 Fig. 2 PMID:6224417

  15. Using a set of GM(1,1) models to predict values of diagnostic symptoms

    NASA Astrophysics Data System (ADS)

    Tabaszewski, Maciej; Cempel, Czeslaw

    2015-02-01

    The main purpose of this study is to develop a methodology of predicting values of vibration symptoms of fan mills in a combined heat and power (CHP) plant. The study was based on grey system theory and GM(1,1) prognostic models with different window sizes for estimating model parameters. Such models have a number of features that are desirable from the point of view of data characteristics collected by the diagnostic system. When using moving window, GM(1,1) models tend to be adaptive. However, selecting an inappropriate window size can result in excessive forecast errors. The present study proposes three possible methods that can be used in automated diagnostic systems to counteract the excessive increase in the forecast error. A comparative analysis of their performance was conducted using data from fan mills in order to select the method which minimises the forecast error.

  16. Interaction between Simian Virus 40 Major Capsid Protein VP1 and Cell Surface Ganglioside GM1 Triggers Vacuole Formation

    PubMed Central

    Luo, Yong; Motamedi, Nasim; Magaldi, Thomas G.; Gee, Gretchen V.; Atwood, Walter J.

    2016-01-01

    ABSTRACT Simian virus 40 (SV40), a polyomavirus that has served as an important model to understand many aspects of biology, induces dramatic cytoplasmic vacuolization late during productive infection of monkey host cells. Although this activity led to the discovery of the virus in 1960, the mechanism of vacuolization is still not known. Pentamers of the major SV40 capsid protein VP1 bind to the ganglioside GM1, which serves as the cellular receptor for the virus. In this report, we show that binding of VP1 to cell surface GM1 plays a key role in SV40 infection-induced vacuolization. We previously showed that SV40 VP1 mutants defective for GM1 binding fail to induce vacuolization, even though they replicate efficiently. Here, we show that interfering with GM1-VP1 binding by knockdown of GM1 after infection is established abrogates vacuolization by wild-type SV40. Vacuole formation during permissive infection requires efficient virus release, and conditioned medium harvested late during SV40 infection rapidly induces vacuoles in a VP1- and GM1-dependent fashion. Furthermore, vacuolization can also be induced by a nonreplicating SV40 pseudovirus in a GM1-dependent manner, and a mutation in BK pseudovirus VP1 that generates GM1 binding confers vacuole-inducing activity. Vacuolization can also be triggered by purified pentamers of wild-type SV40 VP1, but not by GM1 binding-defective pentamers or by intracellular expression of VP1. These results demonstrate that SV40 infection-induced vacuolization is caused by the binding of released progeny viruses to GM1, thereby identifying the molecular trigger for the activity that led to the discovery of SV40. PMID:27006465

  17. Hydrolysis of GM1-ganglioside by human liver β-galactosidase isoenzymes

    PubMed Central

    Ho, Mae Wan; Cheetham, Peter; Robinson, Donald

    1973-01-01

    1. GM1-ganglioside, specifically tritiated in the terminal galactose, was hydrolysed by two forms of `acid' methylumbelliferyl β-galactosidase isolated on gel filtration. 2. Identification of GM1-ganglioside β-galactosidase activity with the `acid' methyl-umbelliferyl β-galactosidases was based on the following: coincident elution profiles on gel filtration; simultaneous inactivation by heat and other treatments; stabilization of both activities by chloride ions; mutual inhibition of hydrolysis by the two substrates. 3. The two isoenzymes (I) and (II) showed general requirements for a mixture of anionic and nonionic detergents in the hydrolysis of the natural substrate. 4. Isoenzyme (I) differed from (II) in molecular size, pH–activity profile, relative resistance to dilution and in sensitivity to various inhibitors. 5. The most significant difference between the isoenzymes is in substrate saturation kinetics: (I) was hyperbolic whereas (II) was sigmoid. The apparent Michaelis constants were 28μm for (I) and 77μm for (II). Isoenzyme (I) was insensitive to GM2-ganglioside whereas (II) was inhibited, consistent with the hypothesis that GM1-ganglioside (and its analogue) acts as modifier in isoenzyme (II) but not in (I). 6. Isoenzyme (I) was membrane-bound whereas (II) was soluble; the former probably represents isoenzyme (II) bound to membrane components, thereby becoming activated. 7. Membranes may serve a dual role in enzyme catalysis involving lipids: as a medium where both enzyme and substrate are effectively concentrated, and as actual activator of enzymes through binding of the latter to specific membrane components. PMID:4774399

  18. Association to HeLa cells and surface behavior of exogenous gangliosides studied with a fluorescent derivative of GM1

    SciTech Connect

    Masserini, M.; Giuliani, A.; Palestini, P.; Acquotti, D.; Pitto, M.; Chigorno, V.; Tettamanti, G. )

    1990-01-23

    Cultured HeLa cells were incubated with pyrene-GM1/{sup 3}H-radiolabeled GM1 ganglioside (1:4 M/M) mixtures for various times. The process of association of pyrene-GM1 with cells was qualitatively and quantitatively the same as that of {sup 3}H-GM1. The pyrene-GM1 and {sup 3}H-GM1 proportions in the various forms of association with cells were similar to that of the starting ganglioside mixture. After 2-h incubation, the association of ganglioside with cells was well established whereas almost no metabolic processing had occurred. During a 24-h incubation, pyrene- and {sup 3}H-GM1 underwent similar metabolic processing and gave rise to catabolic (GM2 and GM3) and anabolic (GDla) derivatives. Fluorescence spectroscopy experiments carried out with the excimer formation technique on subcellular fractions containing plasma membranes showed that exogenous ganglioside was, in part, associated with the cells in a micellar form removable by trypsin treatment, and in part inserted in a seemingly molecular dispersion. Addition of Ca{sup 2+} salts caused aggregation of the ganglioside, as indicated by the increase of the excimer:monomer fluorescence ratio. The phenomenon was Ca{sup 2+} concentration dependent (maximum at 10 mM), and subsequent addition of EDTA has no effect. The saccharide portion of exogenously incorporated pyrene-GM1 was available to interact with external ligands, as shown by its ability to bind cholera toxin whose addition reduced the collision rate among the ganglioside lipid moieties.

  19. Application of Grey Model GM(1, 1) to Ultra Short-Term Predictions of Universal Time

    NASA Astrophysics Data System (ADS)

    Lei, Yu; Guo, Min; Zhao, Danning; Cai, Hongbing; Hu, Dandan

    2016-03-01

    A mathematical model known as one-order one-variable grey differential equation model GM(1, 1) has been herein employed successfully for the ultra short-term (<10days) predictions of universal time (UT1-UTC). The results of predictions are analyzed and compared with those obtained by other methods. It is shown that the accuracy of the predictions is comparable with that obtained by other prediction methods. The proposed method is able to yield an exact prediction even though only a few observations are provided. Hence it is very valuable in the case of a small size dataset since traditional methods, e.g., least-squares (LS) extrapolation, require longer data span to make a good forecast. In addition, these results can be obtained without making any assumption about an original dataset, and thus is of high reliability. Another advantage is that the developed method is easy to use. All these reveal a great potential of the GM(1, 1) model for UT1-UTC predictions.

  20. The Natural History of Juvenile or Subacute GM2 Gangliosidosis: 21 New Cases and Literature Review of 134 Previously Reported

    PubMed Central

    Maegawa, Gustavo H. B.; Stockley, Tracy; Tropak, Michael; Banwell, Brenda; Blaser, Susan; Kok, Fernando; Giugliani, Roberto; Mahuran, Don; Clarke, Joe T. R.

    2010-01-01

    OBJECTIVE Juvenile GM2 gangliosidosis is a group of inherited neurodegenerative diseases caused by deficiency of lysosomal β-hexosaminidase resulting in GM2 ganglioside accumulation in brain. The purpose of this study was to delineate the natural history of the condition and identify genotype-phenotype correlations that might be helpful in predicting the course of the disease in individual patients. METHODS A cohort of 21 patients with juvenile GM2 gangliosidosis, 15 with the Tay-Sachs variant and 6 with the Sandhoff variant, was studied prospectively in 2 centers. Our experience was compared with previously published reports on 134 patients. Information about clinical features, β-hexosaminidase enzyme activity, and mutation analysis was collected. RESULTS In our cohort of patients, the mean (±SD) age of onset of symptoms was 5.3 ± 4.1 years, with a mean follow-up time of 8.4 years. The most common symptoms at onset were gait disturbances (66.7%), incoordination (52.4%), speech problems (28.6%), and developmental delay (28.6%). The age of onset of gait disturbances was 7.1 ± 5.6 years. The mean time for progression to becoming wheelchair-bound was 6.2 ± 5.5 years. The mean age of onset of speech problems was 7.0 ± 5.6 years, with a mean time of progression to anarthria of 5.6 ± 5.3 years. Muscle wasting (10.6 ± 7.4 years), proximal weakness (11.1 ± 7.7 years), and incontinence of sphincters (14.6 ± 9.7 years) appeared later in the course of the disease. Psychiatric disturbances and neuropathy were more prevalent in patients with the Sandhoff variant than in those with the Tay-Sachs variant. However, dysphagia, sphincter incontinence, and sleep problems occurred earlier in those with the Tay-Sachs variant. Cerebellar atrophy was the most common finding on brain MRI (52.9%). The median survival time among the studied and reviewed patients was 14.5 years. The genotype-phenotype correlation revealed that in patients with the Tay-Sachs variant, the presence of R178H and R499H mutations was predictive of an early onset and rapidly progressive course. The presence of either G269S or W474C mutations was associated with a later onset of symptoms along with a more slowly progressive disease course. CONCLUSIONS Juvenile GM2 gangliosidosis is clinically heterogeneous, not only in terms of age of onset and clinical features but also with regard to the course of the disease. In general, the earlier the onset of symptoms, the more rapidly the disease progresses. The Tay-Sachs and Sandhoff variants differed somewhat in the frequency of specific clinical characteristics. Speech deterioration progressed more rapidly than gait abnormalities in both the Tay-Sachs variant and Sandhoff variant groups. Among patients with the Tay-Sachs variant, the HEXA genotype showed a significant correlation with the clinical course. PMID:17015493

  1. Improving the detection of IgM antibodies against glycolipids complexes of GM1 and Galactocerebroside in Multifocal Motor Neuropathy using glycoarray and ELISA assays.

    PubMed

    Delmont, Emilien; Halstead, Susan; Galban-Horcajo, Francesc; Yao, Denggao; Desnuelle, Claude; Willison, Hugh

    2015-01-15

    Antibodies against complexes of GM1:GalC are detected in multifocal motor neuropathy. Previous studies used different techniques, explaining disparities in the results. Antibodies against GM1 and GM1:GalC with different proportions of GalC were measured with both glycoarray and ELISA in 20 multifocal motor neuropathies, and 45 controls. The 1:5 ratio and the 1:1 ratio of GM1:GalC (weight ratio) were respectively the most effective for glycoarray and for ELISA. Testing for anti-GM1:GalC antibodies increased the sensitivity from 40% with anti-GM1 antibodies to 65% with array and 60% with ELISA without loss in specificity (above 91%). Anti-GM1:GalC antibodies are effective biological tools to diagnose multifocal motor neuropathy. PMID:25468269

  2. Synthesis of reference standards to enable single cell metabolomic studies of tetramethylrhodamine-labelled ganglioside GM1

    PubMed Central

    Larsson, E. Andreas; Olsson, Ulf; Whitmore, Colin; Martins, Rita; Tettamanti, Guido; Schnaar, Ronald L.; Dovichi, Norman J.; Palcic, Monica M.; Hindsgaul, Ole

    2007-01-01

    Ganglioside GM1 and its seven potential catabolic products: asialo-GM1, GM2, asialo-GM2, GM3, Lac-Cer, Glc-Cer and Cer, were labelled with tetramethylrhodamine (TMR) to permit ultra-sensitive analysis using laser-induced fluorescence (LIF) detection. The preparation involved acylation of the homogenous C18 lyso-forms of GM1, Lac-Cer, Glc-Cer and Cer with the N-hydroxysuccinimide ester of a β-alanine-tethered 6-TMR derivative, followed by conversion of these labelled products using galactosidase, sialidase and sialyltransferase enzymes. The TMR-glycolipd analogs produced are detectable on TLC down to the 1 ng level by naked eye. All 8 compounds could be separated in under 4 minutes in capillary electrophoresis where they could be detected at the zeptomole (ca 1000 molecule) level using LIF. PMID:17069778

  3. Interfacial stabilization of the antitumoral drug Paclitaxel in monolayers of GM1 and GD1a gangliosides.

    PubMed

    Heredia, Valeria; Maggio, Bruno; Beltramo, Dante M; Dupuy, Fernando G

    2015-10-01

    Molecular interactions between the anti-cancer agent Paclitaxel (Ptx), and two gangliosides with different sialic acid content, GM1 and GD1a, were investigated using the Langmuir film balance technique. Ptx showed interfacial activity reducing the air/water surface tension by 18 mN·m(-1). However, the drug was able to insert into preformed ganglioside monolayers at much higher surface pressures, indicating a preferential interaction of Ptx with GM1 and GD1a. Compression isotherms of binary mixtures of Ptx and GM1 or GD1a also indicated non-ideal mixed monolayers in which the drug became stabilized at the interface in the presence of gangliosides. Ptx reached much higher surface pressure values in the mixed monolayers than those sustained in pure Ptx, although partial desorption of the drug from the interface into the subphase was also observed at high Ptx contents. The mean molecular area of the mixtures showed condensation, mainly in the case of GD1a, whereas Ptx induced a decrease in the compressibility of monolayers when mixed with either GM1 or GD1a. Additionally, Brewster angle microscopy analysis indicated that higher amounts of Ptx are present at the mixed ganglioside/Ptx interface when compared to pure drug monolayers. Finally, GD1a micelles increased in size in the presence of Ptx, whereas GM1 micelles kept their diameter, according to dynamic light scattering measurements, which could be explained by the different properties of ganglioside monolayers. The results obtained on ganglioside-Ptx interactions allowed interpreting the different Ptx loading capacity of GM1 and GD1a, enabling them to act as potential drug carriers. PMID:26119566

  4. Measuring Positive Cooperativity Using the Direct ESI-MS Assay. Cholera Toxin B Subunit Homopentamer Binding to GM1 Pentasaccharide

    NASA Astrophysics Data System (ADS)

    Lin, Hong; Kitova, Elena N.; Klassen, John S.

    2014-01-01

    Direct electrospray ionization mass spectrometry (ESI-MS) assay was used to investigate the stepwise binding of the GM1 pentasaccharide β- D-Gal p-(1→3)-β-D-Gal pNAc-(1→4)[α-D-Neu5Ac-(2→3)]-β- D-Gal p-(1→4)-β-D-Glc p (GM1os) to the cholera toxin B subunit homopentamer (CTB5) and to establish conclusively whether GM1os binding is cooperative. Apparent association constants were measured for the stepwise addition of one to five GM1os to CTB5 at pH 6.9 and 22 °C. The intrinsic association constant, which was established from the apparent association constant for the addition of a single GM1os to CTB5, was found to be (3.2 ± 0.2) × 106 M-1. This is in reasonable agreement with the reported value of (6.4 ± 0.3) × 106 M-1, which was measured at pH 7.4 and 25 °C using isothermal titration calorimetry (ITC). Analysis of the apparent association constants provides direct and unambiguous evidence that GM1os binding exhibits small positive cooperativity. Binding was found to be sensitive to the number of ligand-bound nearest neighbor subunits, with the affinities enhanced by a factor of 1.7 and 2.9 when binding occurs next to one or two ligand-bound subunits, respectively. These findings, which provide quantitative support for the binding model proposed by Homans and coworkers [14], highlight the unique strengths of the direct ESI-MS assay for measuring cooperative ligand binding.

  5. Role of Lipid Rafts and GM1 in the Segregation and Processing of Prion Protein

    PubMed Central

    Botto, Laura; Cunati, Diana; Coco, Silvia; Sesana, Silvia; Bulbarelli, Alessandra; Biasini, Emiliano; Colombo, Laura; Negro, Alessandro; Chiesa, Roberto; Masserini, Massimo; Palestini, Paola

    2014-01-01

    The prion protein (PrPC) is highly expressed within the nervous system. Similar to other GPI-anchored proteins, PrPC is found in lipid rafts, membrane domains enriched in cholesterol and sphingolipids. PrPC raft association, together with raft lipid composition, appears essential for the conversion of PrPC into the scrapie isoform PrPSc, and the development of prion disease. Controversial findings were reported on the nature of PrPC-containing rafts, as well as on the distribution of PrPC between rafts and non-raft membranes. We investigated PrPC/ganglioside relationships and their influence on PrPC localization in a neuronal cellular model, cerebellar granule cells. Our findings argue that in these cells at least two PrPC conformations coexist: in lipid rafts PrPC is present in the native folding (α-helical), stabilized by chemico-physical condition, while it is mainly present in other membrane compartments in a PrPSc-like conformation. We verified, by means of antibody reactivity and circular dichroism spectroscopy, that changes in lipid raft-ganglioside content alters PrPC conformation and interaction with lipid bilayers, without modifying PrPC distribution or cleavage. Our data provide new insights into the cellular mechanism of prion conversion and suggest that GM1-prion protein interaction at the cell surface could play a significant role in the mechanism predisposing to pathology. PMID:24859148

  6. GPR37 Protein Trafficking to the Plasma Membrane Regulated by Prosaposin and GM1 Gangliosides Promotes Cell Viability*

    PubMed Central

    Lundius, Ebba Gregorsson; Vukojević, Vladana; Hertz, Ellen; Stroth, Nikolas; Cederlund, Andreas; Hiraiwa, Masao; Terenius, Lars; Svenningsson, Per

    2014-01-01

    The subcellular distribution of the G protein-coupled receptor GPR37 affects cell viability and is implicated in the pathogenesis of parkinsonism. Intracellular accumulation and aggregation of GPR37 cause cell death, whereas GPR37 located in the plasma membrane provides cell protection. We define here a pathway through which the recently identified natural ligand, prosaposin, promotes plasma membrane association of GPR37. Immunoabsorption of extracellular prosaposin reduced GPR37tGFP surface density and decreased cell viability in catecholaminergic N2a cells. We found that GPR37tGFP partitioned in GM1 ganglioside-containing lipid rafts in the plasma membrane of live cells. This partitioning required extracellular prosaposin and was disrupted by lipid raft perturbation using methyl-β-cyclodextrin or cholesterol oxidase. Moreover, complex formation between GPR37tGFP and the GM1 marker cholera toxin was observed in the plasma membrane. These data show functional association between GPR37, prosaposin, and GM1 in the plasma membrane. These results thus tie together the three previously defined components of the cellular response to insult. Our findings identify a mechanism through which the receptor's natural ligand and GM1 may protect against toxic intracellular GPR37 aggregates observed in parkinsonism. PMID:24371137

  7. Nanodomain Formation of Ganglioside GM1 in Lipid Membrane: Effects of Cholera Toxin-Mediated Cross-Linking.

    PubMed

    Sun, Huijiao; Chen, Licui; Gao, Lianghui; Fang, Weihai

    2015-08-25

    Cross-linking of specific lipid components by proteins mediates transmembrane signaling and material transport. In this work, we conducted coarse-grained simulation to investigate the interactions of binding units of chorela toxin (CTB) with mixed ganglioside GM1 and dipalmitoylphosphatidylcholine (DPPC) lipid bilayer membrane. We determine that the binding of CTB pentamers cross-links GM1 molecules into protein-sized nanodomains that have distinct lipid order compared with the bulk. The toxin in the nanodomain partially penetrates into the membrane. The local disordering can also transmit across the membrane via lipid coupling. Comparison simulations on CTB binding to a membrane that is composed of various lipid components demonstrate that several factors are responsible for the nanodomain formation: (a) the negatively charged headgroup of a GM1 receptor is responsible for the multivalent binding; (b) the head groups being full of hydrogen-bonding donors and receptors stabilize the GM1 cluster itself and ensure the toxin binding with high affinity; and PMID:26250646

  8. The Assembly of GM1 Glycolipid- and Cholesterol-Enriched Raft-Like Membrane Microdomains Is Important for Giardial Encystation

    PubMed Central

    De Chatterjee, Atasi; Mendez, Tavis L.; Roychowdhury, Sukla

    2015-01-01

    Although encystation (or cyst formation) is an important step of the life cycle of Giardia, the cellular events that trigger encystation are poorly understood. Because membrane microdomains are involved in inducing growth and differentiation in many eukaryotes, we wondered if these raft-like domains are assembled by this parasite and participate in the encystation process. Since the GM1 ganglioside is a major constituent of mammalian lipid rafts (LRs) and known to react with cholera toxin B (CTXB), we used Alexa Fluor-conjugated CTXB and GM1 antibodies to detect giardial LRs. Raft-like structures in trophozoites are located in the plasma membranes and on the periphery of ventral discs. In cysts, however, they are localized in the membranes beneath the cyst wall. Nystatin and filipin III, two cholesterol-binding agents, and oseltamivir (Tamiflu), a viral neuraminidase inhibitor, disassembled the microdomains, as evidenced by reduced staining of trophozoites with CTXB and GM1 antibodies. GM1- and cholesterol-enriched LRs were isolated from Giardia by density gradient centrifugation and found to be sensitive to nystatin and oseltamivir. The involvement of LRs in encystation could be supported by the observation that raft inhibitors interrupted the biogenesis of encystation-specific vesicles and cyst production. Furthermore, culturing of trophozoites in dialyzed medium containing fetal bovine serum (which is low in cholesterol) reduced raft assembly and encystation, which could be rescued by adding cholesterol from the outside. Our results suggest that Giardia is able to form GM1- and cholesterol-enriched lipid rafts and these raft domains are important for encystation. PMID:25733521

  9. Clostridium perfringens Alpha-Toxin Induces Gm1a Clustering and Trka Phosphorylation in the Host Cell Membrane

    PubMed Central

    Takagishi, Teruhisa; Oda, Masataka; Kabura, Michiko; Kurosawa, Mie; Tominaga, Kaori; Urano, Shiori; Ueda, Yoshibumi; Kobayashi, Keiko; Kobayashi, Toshihide; Sakurai, Jun; Terao, Yutaka; Nagahama, Masahiro

    2015-01-01

    Clostridium perfringens alpha-toxin elicits various immune responses such as the release of cytokines, chemokines, and superoxide via the GM1a/TrkA complex. Alpha-toxin possesses phospholipase C (PLC) hydrolytic activity that contributes to signal transduction in the pathogenesis of gas gangrene. Little is known about the relationship between lipid metabolism and TrkA activation by alpha-toxin. Using live-cell fluorescence microscopy, we monitored transbilayer movement of diacylglycerol (DAG) with the yellow fluorescent protein-tagged C1AB domain of protein kinase C-γ (EYFP-C1AB). DAG accumulated at the marginal region of the plasma membrane in alpha toxin-treated A549 cells, which also exhibited GM1a clustering and TrkA phosphorylation. Annexin V binding assays showed that alpha-toxin induced the exposure of phosphatidylserine on the outer leaflet of the plasma membrane. However, H148G, a variant toxin which binds cell membrane and has no enzymatic activity, did not induce DAG translocation, GM1a clustering, or TrkA phosphorylation. Alpha-toxin also specifically activated endogenous phospholipase Cγ-1 (PLCγ-1), a TrkA adaptor protein, via phosphorylation. U73122, an endogenous PLC inhibitor, and siRNA for PLCγ-1 inhibited the formation of DAG and release of IL-8. GM1a accumulation and TrkA phosphorylation in A549 cells treated with alpha-toxin were also inhibited by U73122. These results suggest that the flip-flop motion of hydrophobic lipids such as DAG leads to the accumulation of GM1a and TrkA. We conclude that the formation of DAG by alpha-toxin itself (first step) and activation of endogenous PLCγ-1 (second step) leads to alterations in membrane dynamics, followed by strong phosphorylation of TrkA. PMID:25910247

  10. Differential uPAR recruitment in caveolar-lipid rafts by GM1 and GM3 gangliosides regulates endothelial progenitor cells angiogenesis

    PubMed Central

    Margheri, Francesca; Papucci, Laura; Schiavone, Nicola; D'Agostino, Riccardo; Trigari, Silvana; Serratì, Simona; Laurenzana, Anna; Biagioni, Alessio; Luciani, Cristina; Chillà, Anastasia; Andreucci, Elena; Del Rosso, Tommaso; Margheri, Giancarlo; Del Rosso, Mario; Fibbi, Gabriella

    2015-01-01

    Gangliosides and the urokinase plasminogen activator receptor (uPAR) tipically partition in specialized membrane microdomains called lipid-rafts. uPAR becomes functionally important in fostering angiogenesis in endothelial progenitor cells (EPCs) upon recruitment in caveolar-lipid rafts. Moreover, cell membrane enrichment with exogenous GM1 ganglioside is pro-angiogenic and opposite to the activity of GM3 ganglioside. On these basis, we first checked the interaction of uPAR with membrane models enriched with GM1 or GM3, relying on the adoption of solid-supported mobile bilayer lipid membranes with raft-like composition formed onto solid hydrophilic surfaces, and evaluated by surface plasmon resonance (SPR) the extent of uPAR recruitment. We estimated the apparent dissociation constants of uPAR-GM1/GM3 complexes. These preliminary observations, indicating that uPAR binds preferentially to GM1-enriched biomimetic membranes, were validated by identifying a pro-angiogenic activity of GM1-enriched EPCs, based on GM1-dependent uPAR recruitment in caveolar rafts. We have observed that addition of GM1 to EPCs culture medium promotes matrigel invasion and capillary morphogenesis, as opposed to the anti-angiogenesis activity of GM3. Moreover, GM1 also stimulates MAPKinases signalling pathways, typically associated with an angiogenesis program. Caveolar-raft isolation and Western blotting of uPAR showed that GM1 promotes caveolar-raft partitioning of uPAR, as opposed to control and GM3-challenged EPCs. By confocal microscopy, we have shown that in EPCs uPAR is present on the surface in at least three compartments, respectively, associated to GM1, GM3 and caveolar rafts. Following GM1 exogenous addition, the GM3 compartment is depleted of uPAR which is recruited within caveolar rafts thereby triggering angiogenesis. PMID:25313007

  11. GM1 ganglioside reduces the motor incoordination and loss of righting reflex caused by acute ethanol in C57BL/6J mice

    SciTech Connect

    Wallis, C.; Rezazadeh, S.M.; Forster, M.J.; Lal, H. )

    1992-02-26

    Ethanol produces its intoxicating effects by modifying neuronal membranes. Gangliosides stabilize neuronal membranes and promote their recovery from a variety of insults. In this experiment, the efficacy of GM1(i.p.) to reverse ethanol intoxication was evaluated in male mice trained to run on a constantly accelerating rotorod. When mice were tested 15-min following saline or ethanol GM1 pre-treatment reduced rotorod performance by 15% but was ineffective in modifying the ethanol-impaired performance. However, when mice were tested at 15, 35, 55, 75, and 95 min intervals following ethanol, GM1 pre-treatments dose-dependently reduced the efficacy and duration of ethanol in producing motor incoordination. Further, GM1 given prior to ethanol significantly prolonged the time to onset of the loss of righting reflex from 1.4 to 1.9 min, and reduced the duration of the righting-reflex loss from 94 to 77 min. This GM1 effect was seen at 24 h, but not at 48 or 72 h after its administration. The blood ethanol concentration at awakening was significantly higher in 24h GM1-treated animals than in controls suggesting that the GM1 effect was not due to an alteration in ethanol clearance. These findings support the hypothesis that GM1 promotes recovery from ethanol intoxication via a neuroprotective mechanism.

  12. Biochemical characterization of the interactions between doxorubicin and lipidic GM1 micelles with or without paclitaxel loading.

    PubMed

    Leonhard, Victoria; Alasino, Roxana V; Bianco, Ismael D; Garro, Ariel G; Heredia, Valeria; Beltramo, Dante M

    2015-01-01

    Doxorubicin (Dox) is an anthracycline anticancer drug with high water solubility, whose use is limited primarily due to significant side effects. In this study it is shown that Dox interacts with monosialoglycosphingolipid (GM1) ganglioside micelles primarily through hydrophobic interactions independent of pH and ionic strength. In addition, Dox can be incorporated even into GM1 micelles already containing highly hydrophobic paclitaxel (Ptx). However, it was not possible to incorporate Ptx into Dox-containing GM1 micelles, suggesting that Dox could be occupying a more external position in the micelles. This result is in agreement with a higher hydrolysis of Dox than of Ptx when micelles were incubated at alkaline pH. The loading of Dox into GM1 micelles was observed over a broad range of temperature (4°C-55°C). Furthermore, Dox-loaded micelles were stable in aqueous solutions exhibiting no aggregation or precipitation for up to 2 months when kept at 4°C-25°C and even after freeze-thawing cycles. Upon exposure to blood components, Dox-containing micelles were observed to interact with human serum albumin. However, the amount of human serum albumin that ended up being associated to the micelles was inversely related to the amount of Dox, suggesting that both could share their binding sites. In vitro studies on Hep2 cells showed that the cellular uptake and cytotoxic activity of Dox and Ptx from the micellar complexes were similar to those of the free form of these drugs, even when the micelle was covered with albumin. These results support the idea of the existence of different nano-domains in a single micelle and the fact that this micellar model could be used as a platform for loading and delivering hydrophobic and hydrophilic active pharmaceutical ingredients. PMID:26005348

  13. Biochemical characterization of the interactions between doxorubicin and lipidic GM1 micelles with or without paclitaxel loading

    PubMed Central

    Leonhard, Victoria; Alasino, Roxana V; Bianco, Ismael D; Garro, Ariel G; Heredia, Valeria; Beltramo, Dante M

    2015-01-01

    Doxorubicin (Dox) is an anthracycline anticancer drug with high water solubility, whose use is limited primarily due to significant side effects. In this study it is shown that Dox interacts with monosialoglycosphingolipid (GM1) ganglioside micelles primarily through hydrophobic interactions independent of pH and ionic strength. In addition, Dox can be incorporated even into GM1 micelles already containing highly hydrophobic paclitaxel (Ptx). However, it was not possible to incorporate Ptx into Dox-containing GM1 micelles, suggesting that Dox could be occupying a more external position in the micelles. This result is in agreement with a higher hydrolysis of Dox than of Ptx when micelles were incubated at alkaline pH. The loading of Dox into GM1 micelles was observed over a broad range of temperature (4°C–55°C). Furthermore, Dox-loaded micelles were stable in aqueous solutions exhibiting no aggregation or precipitation for up to 2 months when kept at 4°C–25°C and even after freeze–thawing cycles. Upon exposure to blood components, Dox-containing micelles were observed to interact with human serum albumin. However, the amount of human serum albumin that ended up being associated to the micelles was inversely related to the amount of Dox, suggesting that both could share their binding sites. In vitro studies on Hep2 cells showed that the cellular uptake and cytotoxic activity of Dox and Ptx from the micellar complexes were similar to those of the free form of these drugs, even when the micelle was covered with albumin. These results support the idea of the existence of different nano-domains in a single micelle and the fact that this micellar model could be used as a platform for loading and delivering hydrophobic and hydrophilic active pharmaceutical ingredients. PMID:26005348

  14. Identification of flanking SSR markers for a major rice gall midge resistance gene Gm1 and their validation.

    PubMed

    Biradar, S K; Sundaram, R M; Thirumurugan, T; Bentur, J S; Amudhan, S; Shenoy, V V; Mishra, B; Bennett, J; Sarma, N P

    2004-11-01

    Host-plant resistance is the preferred strategy for management of Asian rice gall midge (Orseolia oryzae), a serious pest in many rice-growing countries. The deployment of molecular markers linked to gall midge resistance genes in breeding programmes can accelerate the development of resistant cultivars. In the present study, we have tagged and mapped a dominant gall midge resistance gene, Gm1, from the Oryza sativa cv. W1263 on chromosome 9, using SSR markers. A progeny-tested F2 mapping population derived from the cross W1263/TN1 was used for analysis. To map the gene locus, initially a subset of the F2 mapping population consisting of 20 homozygous resistant and susceptible lines each was screened with 63 parental polymorphic SSR markers. The SSR markers RM316, RM444 and RM219, located on chromosome 9, are linked to Gm1 at genetic distances of 8.0, 4.9 and 5.9 cM, respectively, and flank the gene locus. Further, gene/marker order was also determined. The utility of the co-segregating SSR markers was tested in a backcross population derived from the cross Swarna/W1263//Swarna, and allelic profiles of these markers were analysed in a set of donor rice genotypes possessing Gm1 and in a few gall midge-susceptible, elite rice varieties. PMID:15278284

  15. GM1 and GM3 gangliosides highlight distinct lipid microdomains within the apical domain of epithelial cells.

    PubMed

    Janich, Peggy; Corbeil, Denis

    2007-05-01

    The apical domain of epithelial cells is composed of distinct subdomains such as microvilli, primary cilia and a non-protruding region. Using the cholesterol-binding protein prominin-1 as a specific marker of plasma membrane protrusions we have previously proposed the co-existence of different cholesterol-based lipid microdomains (lipid rafts) within the apical domain [Röper, K., Corbeil, D. and Huttner, W.B. (2000), Retention of prominin in microvilli reveals distinct cholesterol-based lipid microdomains in the apical plasma membrane. Nat. Cell Biol. 2, 582-592]. To substantiate the hypothesis that the microvillar plasma membrane subdomains contain a distinct set of lipids compared to the planar portion we have investigated the distribution of prominin-1 and two raft-associated gangliosides GM(1) and GM(3) by fluorescence microscopy. GM(1) was found to co-localize with prominin-1 on microvilli whereas GM(3) was segregated from there suggesting its localization in the planar region. Regarding the primary cilium, overlapping fluorescent signals of GM(1) or GM(3) and prominin-1 were observed. Thus, our data demonstrate that specific ganglioside-enriched rafts are found in different apical subdomains and reveal that two plasma membrane protrusions with different structural bases (actin for the microvillus and tubulin for the cilium) are composed of distinct types of lipid. PMID:17428477

  16. Single molecule experiments emphasize GM1 as a key player of the different cytotoxicity of structurally distinct Aβ1-42 oligomers.

    PubMed

    Calamai, Martino; Evangelisti, Elisa; Cascella, Roberta; Parenti, Niccoló; Cecchi, Cristina; Stefani, Massimo; Pavone, Francesco

    2016-02-01

    It is well established that cytotoxic Aβ oligomers are the key factor that triggers the initial tissue and cell modifications eventually culminating in the development of Alzheimer's disease. Aβ1-42 oligomers display a high degree of polymorphism, and several structurally different oligomers have been described. Amongst them, two types, recently classified as A+ and A-, have been shown to possess similar size but distinct toxic properties, as a consequence of their biophysical and structural differences. Here, we have investigated by means of single molecule tracking the oligomer mobility on the plasma membrane of living neuroblastoma cells and the interaction with the ganglioside GM1, a component of membrane rafts. We have found that A+ and A- oligomers display a similar lateral diffusion on the plasma membrane of living cells. However, only the toxic A+ oligomers appear to interact and alter the mobility of GM1. We have also studied the lateral diffusion of each kind of oligomers in cells depleted or enriched in GM1. We found that the content of GM1 influences the diffusion of both types of oligomer, although the effect of the increased levels of GM1 is higher for the A+ type. Interestingly, the content of GM1 also affects significantly the mobility of GM1 molecules themselves. PMID:26656159

  17. Binding Cooperativity Matters: A GM1-Like Ganglioside-Cholera Toxin B Subunit Binding Study Using a Nanocube-Based Lipid Bilayer Array

    PubMed Central

    Weatherston, Joshua D.

    2016-01-01

    Protein-glycan recognition is often mediated by multivalent binding. These multivalent bindings can be further complicated by cooperative interactions between glycans and individual glycan binding subunits. Here we have demonstrated a nanocube-based lipid bilayer array capable of quantitatively elucidating binding dissociation constants, maximum binding capacity, and binding cooperativity in a high-throughput format. Taking cholera toxin B subunit (CTB) as a model cooperativity system, we studied both GM1 and GM1-like gangliosides binding to CTB. We confirmed the previously observed CTB-GM1 positive cooperativity. Surprisingly, we demonstrated fucosyl-GM1 has approximately 7 times higher CTB binding capacity than GM1. In order to explain this phenomenon, we hypothesized that the reduced binding cooperativity of fucosyl-GM1 caused the increased binding capacity. This was unintuitive, as GM1 exhibited higher binding avidity (16 times lower dissociation constant). We confirmed the hypothesis using a theoretical stepwise binding model of CTB. Moreover, by taking a mixture of fucosyl-GM1 and GM2, we observed the mild binding avidity fucosyl-GM1 activated GM2 receptors enhancing the binding capacity of the lipid bilayer surface. This was unexpected as GM2 receptors have negligible binding avidity in pure GM2 bilayers. These unexpected discoveries demonstrate the importance of binding cooperativity in multivalent binding mechanisms. Thus, quantitative analysis of multivalent protein-glycan interactions in heterogeneous glycan systems is of critical importance. Our user-friendly, robust, and high-throughput nanocube-based lipid bilayer array offers an attractive method for dissecting these complex mechanisms. PMID:27070150

  18. Ganglioside GM1-mediated transcytosis of cholera toxin bypasses the retrograde pathway and depends on the structure of the ceramide domain.

    PubMed

    Saslowsky, David E; te Welscher, Yvonne M; Chinnapen, Daniel J-F; Wagner, Jessica S; Wan, Joy; Kern, Eli; Lencer, Wayne I

    2013-09-01

    Cholera toxin causes diarrheal disease by binding ganglioside GM1 on the apical membrane of polarized intestinal epithelial cells and trafficking retrograde through sorting endosomes, the trans-Golgi network (TGN), and into the endoplasmic reticulum. A fraction of toxin also moves from endosomes across the cell to the basolateral plasma membrane by transcytosis, thus breeching the intestinal barrier. Here we find that sorting of cholera toxin into this transcytotic pathway bypasses retrograde transport to the TGN. We also find that GM1 sphingolipids can traffic from apical to basolateral membranes by transcytosis in the absence of toxin binding but only if the GM1 species contain cis-unsaturated or short acyl chains in the ceramide domain. We found previously that the same GM1 species are needed to efficiently traffic retrograde into the TGN and endoplasmic reticulum and into the recycling endosome, implicating a shared mechanism of action for sorting by lipid shape among these pathways. PMID:23884419

  19. Naturally occurring GM2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B GM2 gangliosidosis).

    PubMed

    Fox, J; Li, Y T; Dawson, G; Alleman, A; Johnsrude, J; Schumacher, J; Homer, B

    1999-01-01

    Two juvenile sibling male Muntjak deer (Muntiacus muntjak) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the brain, ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxolfast blue stains. Within the brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the brain and retina revealed numerous neurons and ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of G(M2) ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl beta-GlcNAc, but not 4-methylumbelliferyl beta-GlcNAc-6-sulfate, indicating the defect of beta-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of beta-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B G(M2) gangliosidosis (classical Tay-Sachs disease). PMID:9930895

  20. Late onset GM2 gangliosidosis mimicking spinal muscular atrophy.

    PubMed

    Jamrozik, Z; Lugowska, A; Gołębiowski, M; Królicki, L; Mączewska, J; Kuźma-Kozakiewicz, M

    2013-09-25

    A case of late onset GM2 gangliosidodis with spinal muscular atrophy phenotype followed by cerebellar and extrapyramidal symptoms is presented. Genetic analysis revealed compound heterozygous mutation in exon 10 of the HEXA gene. Patient has normal intelligence and emotional reactivity. Neuroimaging tests of the brain showed only cerebellar atrophy consistent with MR spectroscopy (MRS) abnormalities. (18)F-fluorodeoxyglucose positron emission tomography (18)F-FDG PET/CT of the brain revealed glucose hypometabolism in cerebellum and in temporal and occipital lobes bilaterally. PMID:23820084

  1. Comparison of two GM1-erythrocyte assays to detect heat-labile Escherichia coli enterotoxin in stool specimens.

    PubMed

    Germani, Y; Guesdon, J L; Phalente, L; Begaud, E; Moreau, J P

    1988-05-01

    Two erythrocyte immunoassay techniques to detect the presence of Escherichia coli heat-labile enterotoxin (LTh) in stool supernatants and cell-free culture supernatants were compared. In the competitive assay, GM1 ganglioside was coated onto V-shaped-well microdilution plates and enterotoxin was coupled to sheep erythrocytes. As little as 0.8 ng of LTh per ml was detected by this method, which was based on the competition between the LTh of the test sample and the sensitized erythrocytes. The second assay made use of chimera antibody prepared by coupling polyclonal anti-LTh antibody to a monoclonal antibody specific for sheep erythrocytes. In this case, LTh, which was specifically bound to a GM1 ganglioside-coated plate, was detected by successively adding the chimera antibody and sheep erythrocytes. The limit of detection of the chimera antibody erythrocyte immunoassay was 0.2 ng/ml. Stool samples were collected from 167 infants hospitalized for diarrhea in the hospital of Noumea, New Caledonia. False-negative reactions due to proteases present in the stool samples were avoided by the addition of phenylmethylsulfonyl fluoride. PMID:3290242

  2. [Molecular pathogenesis and therapeutic approach of GM2 gangliosidosis].

    PubMed

    Tsuji, Daisuke

    2013-01-01

    Tay-Sachs and Sandhoff diseases (GM2 gangliosidoses) are autosomal recessive lysosomal storage diseases caused by gene mutations in HEXA and HEXB, each encoding human lysosomal β-hexosaminidase α-subunits and β-subunits, respectively. In Tay-Sachs disease, excessive accumulation of GM2 ganglioside (GM2), mainly in the central nervous system, is caused by a deficiency of the HexA isozyme (αβ heterodimer), resulting in progressive neurologic disorders. In Sandhoff disease, combined deficiencies of HexA and HexB (ββ homodimer) cause not only the accumulation of GM2 but also of oligosaccharides carrying terminal N-acetylhexosamine residues (GlcNAc-oligosaccharides), resulting in systemic manifestations including hepatosplenomegaly as well as neurologic symptoms. Hence there is little clinically effective treatment for these GM2 gangliosidoses. Recent studies on the molecular pathogenesis in Sandhoff disease patients and disease model mice have shown the involvement of microglial activation and chemokine induction in neuroinflammation and neurodegeneration in this disease. Experimental and therapeutic approaches, including recombinant enzyme replacement, have been performed using Sandhoff disease model mice, suggesting the future application of novel techniques to treat GM2 gangliosidoses (Hex deficiencies), including Sandhoff disease as well as Tay-Sachs disease. In this study, we isolated astrocytes and microglia from the neonatal brain of Sandhoff disease model mice and demonstrated abnormalities of glial cells. Moreover, we demonstrated the therapeutic effect of an intracerebroventricular administration of novel recombinant human HexA carrying a high content of M6P residue in Sandhoff disease model mice. PMID:23370522

  3. A Trp474Cys mutation in the alpha-subunit of beta-hexosaminidase causes a subacute encephalopathic form of G{sub M2} gangliosidosis, type 1

    SciTech Connect

    Petroulakis, E.; Cao, Z.; Salo, T.

    1994-09-01

    Mutations in the HEXA gene that encodes the {alpha}-subunit of the heterodimeric lysosomal enzyme {beta}-hexosaminidase A, or Hex A ({alpha}{beta}), cause G{sub M2} gangliosidosis, type 1. The infantile form (Tay-Sachs disease) results when there is no residual Hex A activity, while less severe and more variable clinical phenotypes result when residual Hex A activity is present. A non-Jewish male who presented with an acute psychotic episode at age 16 was diagnosed with a subacute encephalopathic form of G{sub M2} gangliosidosis. At age 19, chronic psychosis with intermittent acute exacerbations remains the most disabling symptom in this patient and his affected brother although both exhibit some ataxia and moderately severe dysarthria. We have found a 4 bp insertion (+TATC 1278) associated with infantile Tay-Sachs disease on one allele; no previously identified mutation was found on the second allele. SSCP analysis detected a shift in exon 13 and sequencing revealed a G1422C mutation in the second allele that results in a Trp474Cys substitution. The presence of the mutation was confirmed by the loss of HaeIII and ScrFI sites in exon 13 PCR products from the subjects and their father. The mutation was introduced into the {alpha}-subunit cDNA and Hex S ({alpha}{alpha}) and Hex A ({alpha}{beta}) were transiently expressed in monkey COS-7 cells. The Trp474Cys mutant protein had approximately 5% and 12% of wild-type Hex S and Hex A activity, respectively. Western blot analysis revealed a small amount of residual mature {alpha}-subunit and a normal level of precursor protein. We conclude that the Trp474Cys mutation is the cause of the Hex A deficiency associated with a subacute (juvenile-onset) phenotype in this patient. Like other mutations in exon 13 of HEXA, it appears to affect intracellular processing. Studies of the defect in intracellular processing are in progress.

  4. GM2 gangliosidosis associated with a HEXA missense mutation in Japanese Chin dogs: a potential model for Tay Sachs disease.

    PubMed

    Sanders, Douglas N; Zeng, Rong; Wenger, David A; Johnson, Gary S; Johnson, Gayle C; Decker, Jared E; Katz, Martin L; Platt, Simon R; O'Brien, Dennis P

    2013-01-01

    GM2 gangliosidosis is a fatal lysosomal storage disease caused by a deficiency of β-hexosaminidase (EC 3.2.1.52). There are two major isoforms of the enzyme: hexosaminidase A composed of an α and a β subunit (encoded by HEXA and HEXB genes, respectively); and, hexosaminidase B composed of two β subunits. Hexosaminidase A requires an activator protein encoded by GM2A to catabolize GM2 ganglioside, but even in the absence of the activator protein, it can hydrolyze the synthetic substrates commonly used to assess enzyme activity. GM2 gangliosidosis has been reported in Japanese Chin dogs, and we identified the disease in two related Japanese Chin dogs based on clinical signs, histopathology and elevated brain GM2 gangliosides. As in previous reports, we found normal or elevated hexosaminidase activity when measured with the synthetic substrates. This suggested that the canine disease is analogous to human AB variant of G(M2) gangliosidosis, which results from mutations in GM2A. However, only common neutral single nucleotide polymorphisms were found upon sequence analysis of the canine ortholog of GM2A from the affected Japanese Chins. When the same DNA samples were used to sequence HEXA, we identified a homozygous HEXA:c967G>A transition which predicts a p.E323K substitution. The glutamyl moiety at 323 is known to make an essential contribution to the active site of hexosaminidase A, and none of the 128 normal Japanese Chins and 92 normal dogs of other breeds that we tested was homozygous for HEXA:c967A. Thus it appears that the HEXA:c967G>A transition is responsible for the GM2 gangliosidosis in Japanese Chins. PMID:23266199

  5. Differential Anatomical Expression of Ganglioside GM1 Species Containing d18:1 or d20:1 Sphingosine Detected by MALDI Imaging Mass Spectrometry in Mature Rat Brain

    PubMed Central

    Weishaupt, Nina; Caughlin, Sarah; Yeung, Ken K.-C.; Whitehead, Shawn N.

    2015-01-01

    GM1 ganglioside plays a role in essential neuronal processes, including differentiation, survival, and signaling. Yet, little is known about GM1 species with different sphingosine bases, such as the most abundant species containing 18 carbon atoms in the sphingosine chain (GM1d18:1), and the less abundant containing 20 carbon atoms (GM1d20:1). While absent in the early fetal brain, GM1d20:1 continues to increase throughout pre- and postnatal development and into old age, raising questions about the functional relevance of the GM1d18:1 to GM1d20:1 ratio. Matrix-assisted laser desorption/ionization imaging mass spectrometry is a novel technology that allows differentiation between these two GM1 species and quantification of their expression within an anatomical context. Using this technology, we find GM1d18:1/d20:1 expression ratios are highly specific to defined anatomical brain regions in adult rats. Thus, the ratio was significantly different among different thalamic nuclei and between the corpus callosum and internal capsule. Differential GM1d18:1/GM1d20:1 ratios measured in hippocampal subregions in rat brain complement previous studies conducted in mice. Across layers of the sensory cortex, opposing expression gradients were found for GM1d18:1 and GM1d20:1. Superficial layers demonstrated lower GM1d18:1 and higher GM1d20:1 signal than other layers, while in deep layers GM1d18:1 expression was relatively high and GM1d20:1 expression low. By far the highest GM1d18:1/d20:1 ratio was found in the amygdala. Differential expression of GM1 with d18:1- or d20:1-sphingosine bases in the adult rat brain suggests tight regulation of expression and points toward a distinct functional relevance for each of these GM1 species in neuronal processes. PMID:26648849

  6. The restriction mechanism of Chinese university results efficiency based on the hybrid of VPRS and optimized GM(1,1)

    NASA Astrophysics Data System (ADS)

    Jian, Lirong; Liu, Sifeng

    2013-10-01

    Grey system theory and rough set theory are two different mathematical tools which are used to deal with uncertain or incomplete information, and yet they are relevant and complementary to a certain degree. They both improve the generality of data presentation by reducing its accuracy. In the paper, based on the statistics of Chinese universities science and technology from 2002 year to 2011 year, the key factors which affect the transfer performance are figured out by applying variable precision rough set models, output performance and transfer performance are predicted by optimal GM(1,1) model. According to the above, the restriction mechanism of university R&D performance is analyzed from a whole view of operating model and key influencing factors, and the prospect model is suggested.

  7. Neurotropin promotes NGF signaling through interaction of GM1 ganglioside with Trk neurotrophin receptor in PC12 cells.

    PubMed

    Fukuda, Yu; Fukui, Takao; Hikichi, Chika; Ishikawa, Tomomasa; Murate, Kenichiro; Adachi, Takeshi; Imai, Hideki; Fukuhara, Koki; Ueda, Akihiro; Kaplan, Allen P; Mutoh, Tatsuro

    2015-01-30

    Activation of the high-affinity nerve growth factor (NGF) receptor Trk occurs through multiple processes consisted of translocation and clustering within the plasma membrane lipid rafts, dimerization and autophosphorylation. Here we found that a nonprotein extract of inflamed rabbit skin inoculated with vaccinia virus (Neurotropin(®)) enhanced efficiency of NGF signaling. In rat pheochromocytoma PC12 cells overexpressing Trk (PCtrk cells), Neurotropin augmented insufficient neurite outgrowth observed at suboptimal concentration of NGF (2ng/mL) in a manner depending on Trk kinase activity. Cellular exposure to Neurotropin resulted in an accumulation of Trk-GM1 complexes without affecting dimerization or phosphorylation states of Trk. Following NGF stimulation, Neurotropin significantly facilitated the time course of NGF-induced Trk autophosphorylation. These observations provide a unique mechanism controlling efficiency of NGF signaling, and raise the therapeutic potential of Neurotropin for various neurological conditions associated with neurotrophin dysfunction. PMID:25454796

  8. Binding of CFA/I Pili of Enterotoxigenic Escherichia coli to Asialo-GM1 Is Mediated by the Minor Pilin CfaE.

    PubMed

    Madhavan, T P Vipin; Riches, James D; Scanlon, Martin J; Ulett, Glen C; Sakellaris, Harry

    2016-05-01

    CFA/I pili are representatives of a large family of related pili that mediate the adherence of enterotoxigenic Escherichia coli to intestinal epithelial cells. They are assembled via the alternate chaperone-usher pathway and consist of two subunits, CfaB, which makes up the pilus shaft and a single pilus tip-associated subunit, CfaE. The current model of pilus-mediated adherence proposes that CFA/I has two distinct binding activities; the CfaE subunit is responsible for binding to receptors of unknown structure on erythrocyte and intestinal epithelial cell surfaces, while CfaB binds to various glycosphingolipids, including asialo-GM1. In this report, we present two independent lines of evidence that, contrary to the existing model, CfaB does not bind to asialo-GM1 independently of CfaE. Neither purified CfaB subunits nor CfaB assembled into pili bind to asialo-GM1. Instead, we demonstrate that binding activity toward asialo-GM1 resides in CfaE and this is essential for pilus binding to Caco-2 intestinal epithelial cells. We conclude that the binding activities of CFA/I pili for asialo-GM1, erythrocytes, and intestinal cells are inseparable, require the same amino acid residues in CfaE, and therefore depend on the same or very similar binding mechanisms. PMID:26975993

  9. Penner's serotype 4 of Campylobacter jejuni has a lipopolysaccharide that bears a GM1 ganglioside epitope as well as one that bears a GD1 a epitope.

    PubMed Central

    Yuki, N; Taki, T; Takahashi, M; Saito, K; Tai, T; Miyatake, T; Handa, S

    1994-01-01

    The carbohydrate structures of lipopolysaccharides (LPSs) of Campylobacter jejuni strains belonging to Penner's serotypes (PEN) 1, 2, 4, 19, 23, and 36 were studied by thin-layer chromatography and immunostaining with several monoclonal antiganglioside antibodies. Anti-GM1 and anti-GD1a antibodies reacted with the LPSs of PEN 1, 4, and 19. Aspinall et al. (G. O. Aspinall, A. G. McDonald, T. S. Raju, H. Pang, A. P. Moran, and J. L. Penner. Eur. J. Biochem. 213:1017-1027, 1993) recently reported that the LPS of PEN 4 has a GD1a ganglioside-like structure rather than a GM1-like structure. We found that the LPS fraction of C. jejuni (PEN 4) has an LPS that bears a GM1 epitope as well as an LPS that bears a GD1a epitope. Images PMID:7513313

  10. Selection of Escherichia coli heat-labile toxin (LT) inhibitors using both the GM1-ELISA and the cAMP Vero cell assay.

    PubMed

    Verhelst, Roderick; Schroyen, Martine; Buys, Nadine; Niewold, Theo

    2013-07-01

    Weaned piglets are very susceptible to diarrhea caused by enterotoxigenic Escherichia coli. In the past, various natural components were proposed to have beneficial effects by reducing the effects of diarrheal infectious diseases in humans and animals, and thus may represent an alternative for the use of (prophylactic) antibiotics. Alternatives may inactivate enterotoxigenic Escherichia coli heat-labile toxin (LT) by interfering with toxin binding to the cellular receptor GM1. In this study, various plants and other natural substances were tested for inhibitory properties, in the GM1 binding assay, and in the LT-induced cAMP production in Vero cells. The toxic dose of each compound was determined in a cell viability assay, and the highest nontoxic concentrations were used in the GM1 and cAMP assays. Results demonstrated that only d-(+)-galactose, lactose, N-acetyl-d-galactosamine, and two tea extracts were able to inhibit the binding of LT to its GM1 receptor. In the cAMP assay, only the two tea extracts showed inhibitory activity. This shows that d-(+)-galactose, lactose, and N-acetyl-d-galactosamine can indeed inhibit LT binding to GM1 based on structural homology with GM1 in the absence of living cells. However, in the cAMP assay, d-(+)-galactose, and lactose, N-acetyl-d-galactosamine are apparently metabolized to below their effective inhibitory concentration, likely predicting limited practical applicability in vivo. Both tea extracts maintained their activity in the presence of cells. The active compounds in both are probably polyphenols, which are not easily metabolized, and most likely work by aggregating the toxin. In conclusion, the combination of methods used here is a convenient and fast method for preselecting natural substances containing potentially toxin-binding compounds. Furthermore, if antidiarrhea activity is attributed to compounds found inactive here, their activity is unlikely based on interference with toxin binding. PMID:23692076

  11. Chicken egg yolk anti-asialoGM1 immunoglobulin (IgY): an inexpensive glycohistochemical probe for localization of T-antigen in human colorectal adenocarcinomas.

    PubMed

    Sriram, V; Jebaraj, C E; Yogeeswaran, G

    1999-07-01

    A egg yolk polyclonal IgY has been prepared by immunization of white leghorn chickens with small unilamellar liposomal asialoGM1. The newly prepared anti-asialoGM1 IgY has been characterized to be specific toward the terminal carbohydrate moiety of asialoGM1, and has no cross reactivity to its sialylated counterpart (ganglioside, GM1) as evidenced by immunochromatographic studies. General glycohistochemical methods along with antigen specific lectin and immunohistochemical staining using anti-asialoGM1 IgY were used to study the expression of Thomsen-Friedenreich (T-) disaccharide antigen in human colorectal adenocarcinoma tissues. The expression of T-antigen in colon cancer tissue was detected by two T-disaccharide specific probes, chicken anti-T-yolk antibody (IgY) and Artocarpus integrifolia lectin (AIL) and was found to be more pronounced in both the secreted mucin as well as the cytoplasmic mucin deposits. These immunochemical detection methods for T-antigen showed a weaker correlation with other glycostaining methods using, alcian-blue/periodic acid-Schiff (AB-PAS) and high iron diamine (HID). However, a general enzymatic staining for galactose and galactosamine containing glycoconjugates, by galactose oxidase-Schiff method, showed a good correlation with T-antigen detection. While the T-beta specific anti-asialoGM1 could localize T-antigen in 11 of 13 (84%) human colorectal adenocarcinoma tissue sections tested, the T-alpha specific AIL could localize the T-antigen in only 6 of the tissues (46%). These observations confirm previously reported findings, of the prevalence of T-beta conformation in colon cancer, that binds significantly more with the anti-asialoGM1 IgY than with the T-alpha specific AIL. Hence, both anti-T IgY and the AIL immunohistochemical probes may have useful diagnostic value because of the ease of preparation and cost effectiveness, but the T-beta specific anti-asialoGM1 probe (IgY) would have a better prognostic value in colon adenocarcinomas. PMID:10522151

  12. Polycystic kidneys and GM2 gangliosidosis-like disease in neonatal springboks (Antidorcas marsupialis).

    PubMed

    Herder, V; Kummrow, M; Leeb, T; Sewell, A C; Hansmann, F; Lehmbecker, A; Wohlsein, P; Baumgärtner, W

    2015-05-01

    Clinical, gross, histopathologic, electron microscopic findings and enzymatic analysis of 4 captive, juvenile springboks (Antidorcas marsupialis) showing both polycystic kidneys and a storage disease are described. Springbok offspring (4 of 34; 12%) were affected by either one or both disorders in a German zoo within a period of 5 years (2008-2013). Macroscopic findings included bilaterally severely enlarged kidneys displaying numerous cysts in 4 animals and superior brachygnathism in 2 animals. Histopathologically, kidneys of 4 animals displayed cystic dilation of the renal tubules. In addition, abundant cytoplasmic vacuoles with a diameter ranging from 2 to 10 μm in neurons of the central and peripheral nervous system, hepatocytes, thyroid follicular epithelial cells, pancreatic islets of Langerhans and renal tubular cells were found in 2 springbok neonates indicative of an additional storage disease. Ultrastructurally, round electron-lucent vacuoles, up to 4 μm in diameter, were present in neurons. Enzymatic analysis of liver and kidney tissue of 1 affected springbok revealed a reduced activity of total hexosaminidase (Hex) with relatively increased HexA activity at the same level of total Hex, suggesting a hexosaminidase defect. Pedigree analysis suggested a monogenic autosomal recessive inheritance for both diseases. In summary, related springboks showed 2 different changes resembling both polycystic kidney and a GM2 gangliosidosis similar to the human Sandhoff disease. Whether the simultaneous occurrence of these 2 entities represents an incidental finding or has a genetic link needs to be investigated in future studies. PMID:25232033

  13. Protease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis model.

    PubMed

    Kitakaze, Keisuke; Mizutani, Yasumichi; Sugiyama, Eiji; Tasaki, Chikako; Tsuji, Daisuke; Maita, Nobuo; Hirokawa, Takatsugu; Asanuma, Daisuke; Kamiya, Mako; Sato, Kohei; Setou, Mitsutoshi; Urano, Yasuteru; Togawa, Tadayasu; Otaka, Akira; Sakuraba, Hitoshi; Itoh, Kohji

    2016-05-01

    GM2 gangliosidoses, including Tay-Sachs and Sandhoff diseases, are neurodegenerative lysosomal storage diseases that are caused by deficiency of β-hexosaminidase A, which comprises an αβ heterodimer. There are no effective treatments for these diseases; however, various strategies aimed at restoring β-hexosaminidase A have been explored. Here, we produced a modified human hexosaminidase subunit β (HexB), which we have termed mod2B, composed of homodimeric β subunits that contain amino acid sequences from the α subunit that confer GM2 ganglioside-degrading activity and protease resistance. We also developed fluorescent probes that allow visualization of endocytosis of mod2B via mannose 6-phosphate receptors and delivery of mod2B to lysosomes in GM2 gangliosidosis models. In addition, we applied imaging mass spectrometry to monitor efficacy of this approach in Sandhoff disease model mice. Following i.c.v. administration, mod2B was widely distributed and reduced accumulation of GM2, asialo-GM2, and bis(monoacylglycero)phosphate in brain regions including the hypothalamus, hippocampus, and cerebellum. Moreover, mod2B administration markedly improved motor dysfunction and a prolonged lifespan in Sandhoff disease mice. Together, the results of our study indicate that mod2B has potential for intracerebrospinal fluid enzyme replacement therapy and should be further explored as a gene therapy for GM2 gangliosidoses. PMID:27018595

  14. Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNA.

    PubMed

    Wicklow, Brandy A; Ivanovich, Jennifer L; Plews, Margot M; Salo, Timothy J; Noetzel, Michael J; Lueder, Gregg T; Cartegni, Luca; Kaback, Michael M; Sandhoff, Konrad; Steiner, Robert D; Triggs-Raine, Barbara L

    2004-06-01

    We have characterized the molecular basis of beta-hexosaminidase A (HEX A) deficiency in a patient ascertained through an ophthalmologic examination that revealed cherry red spots on his retina. The absence of neurological deficit in this child until 3 3/4 years of age indicated residual HEX A must be present. Three HEXA mutations, 10T > C (S4P) and 972T > A (V324V) on the maternal allele, and 1A > T (M1L) on the paternal allele were identified. The effects of the amino acid substitutions on HEX A expressed in COS-7 cells were analyzed; as expected, no HEX A activity was associated with the M1L mutation but surprisingly, the S4P mutation resulted in 59% of the HEX A activity expressed by the wild type cDNA. The effect of the S4P change was much less than that of another HEXA mutation, G269S, associated with an adult onset form of G(M2) gangliosidosis. This indicated that the S4P change was not the cause of disease and suggested that one of the mutations on the maternal allele, 10T > C or 972T > A, had its effect at the mRNA level. This was confirmed by Northern blot analysis that showed only 7% of the normal level of HEXA mRNA in proband fibroblasts. Analysis of the residual mRNA by RT/PCR and sequencing revealed normal transcripts from both the maternal and paternal allele, as well as a low abundance aberrant transcript from the maternal allele. Sequencing of this aberrant transcript revealed a new exon 8 donor site created by the 972T > A mutation that resulted in a 17 bp deletion and destabilization of the resulting abnormal transcript. The remaining normal mRNA produced from the 972T > A allele must account for the delayed onset of clinical symptoms in this child. PMID:15108204

  15. Stem Cell Transplant for Inborn Errors of Metabolism

    ClinicalTrials.gov

    2012-11-06

    Adrenoleukodystrophy; Metachromatic Leukodystrophy; Globoid Cell Leukodystrophy; Gaucher's Disease; Fucosidosis; Wolman Disease; Niemann-Pick Disease; Batten Disease; GM1 Gangliosidosis; Tay Sachs Disease; Sandhoff Disease

  16. Meningoencephalitis-like onset of post-infectious AQP4-IgG-positive optic neuritis complicated by GM1-IgG-positive acute polyneuropathy.

    PubMed

    Benedetti, Luana; Franciotta, Diego; Beronio, Alessandro; Delucchi, Stefano; Capellini, Cesare; Del Sette, Massimo

    2015-02-01

    Fifteen days after a respiratory infection, a 45-year-old woman presented with paresthesias in the hands and feet, bilateral loss of vision, fever, headache, and impairment of consciousness. Magnetic resonance imaging (MRI) showed predominant lesions in the optic tracts, optic chiasm, and hypothalamus. Cerebrospinal fluid analysis revealed elevated protein level, and lymphocytic pleocytosis. Neurophysiological studies disclosed a demyelinating sensorimotor polyneuropathy. Serum anti-Mycoplasma pneumoniae immunoglobulin (Ig)M, anti-GM1 IgG, and anti-AQP4 IgG were positive. This case, which is remarkable for post-infectious meningoencephalitis-like onset, MRI picture, and dysimmunity to central and peripheral nervous system autoantigens, underpins the pivotal diagnostic role of anti-AQP4-IgG, and expands the list of clinico-pathological findings that can associate with neuromyelitis optica spectrum disorders. PMID:24557856

  17. Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver.

    PubMed Central

    Pohlentz, G; Klein, D; Schwarzmann, G; Schmitz, D; Sandhoff, K

    1988-01-01

    Competition experiments using lactosylceramide, ganglioside GM3 and ganglioside GD3 as substrates, as well as mutual inhibitors for ganglioside N-acetylgalactosaminyltransferase, in Golgi vesicles derived from rat liver suggested that N-acetylgalactosamine transfer to these three respective compounds, leading to gangliosides GA2, GM2, and GD2, respectively, is catalyzed by one enzyme. Analogous studies with gangliosides GA1, GM1, and GD1b as glycolipid acceptors in sialyltransferase assays indicated GM1b, GD1a, and GT1b synthases to be identical. These results are incorporated into a model for ganglioside biosynthesis and its regulation. PMID:3140234

  18. High-Speed Single-Particle Tracking of GM1 in Model Membranes Reveals Anomalous Diffusion due to Interleaflet Coupling and Molecular Pinning

    PubMed Central

    2015-01-01

    The biological functions of the cell membrane are influenced by the mobility of its constituents, which are thought to be strongly affected by nanoscale structure and organization. Interactions with the actin cytoskeleton have been proposed as a potential mechanism with the control of mobility imparted through transmembrane “pickets” or GPI-anchored lipid nanodomains. This hypothesis is based on observations of molecular mobility using various methods, although many of these lack the spatiotemporal resolution required to fully capture all the details of the interaction dynamics. In addition, the validity of certain experimental approaches, particularly single-particle tracking, has been questioned due to a number of potential experimental artifacts. Here, we use interferometric scattering microscopy to track molecules labeled with 20–40 nm scattering gold beads with simultaneous <2 nm spatial and 20 μs temporal precision to investigate the existence and mechanistic origin of anomalous diffusion in bilayer membranes. We use supported lipid bilayers as a model system and demonstrate that the label does not influence time-dependent diffusion in the small particle limit (≤40 nm). By tracking the motion of the ganglioside lipid GM1 bound to the cholera toxin B subunit for different substrates and lipid tail properties, we show that molecular pinning and interleaflet coupling between lipid tail domains on a nanoscopic scale suffice to induce transient immobilization and thereby anomalous subdiffusion on the millisecond time scale. PMID:25133992

  19. Electrokinetic and electrostatic properties of bilayers containing gangliosides GM1, GD1a, or GT1. Comparison with a nonlinear theory.

    PubMed Central

    McDaniel, R V; Sharp, K; Brooks, D; McLaughlin, A C; Winiski, A P; Cafiso, D; McLaughlin, S

    1986-01-01

    We formed vesicles from mixtures of egg phosphatidylcholine (PC) and the gangliosides GM1, GD1a, or GT1 to model the electrokinetic properties of biological membranes. The electrophoretic mobilities of the vesicles are similar in NaCl, CsCl, and TMACl solutions, suggesting that monovalent cations do not bind significantly to these gangliosides. If we assume the sialic acid groups on the gangliosides are located some distance from the surface of the vesicle and the sugar moieties exert hydrodynamic drag, we can describe the mobility data in 1, 10, and 100 mM monovalent salt solutions with a combination of the Navier-Stokes and nonlinear Poisson-Boltzmann equations. The values we assume for the thickness of the ganglioside head group and the location of the charge affect the theoretical predictions markedly, but the Stokes radius of each sugar and the location of the hydrodynamic shear plane do not. We obtain a reasonable fit to the mobility data by assuming that all ganglioside head groups project 2.5 nm from the bilayer and all fixed charges are in a plane 1 nm from the bilayer surface. We tested the latter assumption by estimating the surface potentials of PC/ganglioside bilayers using four techniques: we made 31P nuclear magnetic resonance, fluorescence, electron spin resonance, and conductance measurements. The results are qualitatively consistent with our assumption. PMID:3697476

  20. EXPERIENTIAL FACTORS IN THE EXPRESSION OF HYPERMOTILITY PRODUCED BY INTRADENTATE COLCHICINE: LACK OF EFFECT OF GM1 GANGLIOSIDE ON COLCHICINE-INDUCED LOSS OF GRANULE CELLS AND MOSSY FIBERS (JOURNAL VERSION)

    EPA Science Inventory

    Adult, male Fischer-344 rats were given bilateral injections of 2.5 microgram colchicine or artificial cerebrospinal fluid into caudal and rostral sites of the dentate gyrus of the hippocampus. One group of rats received 21 consecutive daily injections of 20 mg/kg GM1 ganglioside...

  1. Aggregation of liposomes induced by the toxic peptides Alzheimer's Abetas, human amylin and prion (106-126): facilitation by membrane-bound GM1 ganglioside.

    PubMed

    Kurganov, Boris; Doh, Michael; Arispe, Nelson

    2004-02-01

    To compare both the peptide molecular self-aggregation and the interaction with membrane lipids of the Alzheimer's amyloid beta (Abeta)40, Abeta42 peptides, and the cytotoxic peptides human amylin and prion (106-126) peptides, we applied a liposome aggregation technology. The kinetics of the changes in the optical density (DeltaOD) of liposome suspensions generated by the aggregation of liposomes induced by these peptides, allowed us to comparatively analyze their phospholipid affinity and self-aggregation. The kinetic curves showed an initial nonlinear region where d(DeltaOD)/dt followed first order kinetics corresponding to the binding of the peptides to the membrane of the liposome, a linear region where d(DeltaOD)/dt was constant, corresponding to the interaction between two membrane-bound peptide molecules, and a final slower increasing nonlinear region that corresponds to nucleation or seeding of aggregation. The analysis of the aggregation curves demonstrated that amylin and prion peptides also showed affinity for the acidic phospholipid phosphatidylserine (PS), as it has previously been shown for the Alzheimer's Abeta40, Abeta42 peptides. Abeta42 showed the highest, and amylin the lowest, affinity for the liposome membrane. When bound to the membrane of the liposomes, all the peptides preserved the self-aggregation characteristics observed in solution. Aging the Abeta40 and Abeta42 peptide solutions that permit molecular self-aggregation reduced their capacity to induce liposome aggregation. The self-aggregation of membrane-bound prion molecules was several orders of magnitude higher than that observed for the other toxic peptides. Incorporation of the ganglioside GM1 into the membrane of liposomes enhanced the peptide-induced liposome aggregation. Kinetic analysis revealed that this enhancement was due to facilitation of the formation of bridges between membrane-bound peptide molecules, demonstrating that the peptide-membrane interaction and the peptide amyloidogenesis are independent functions performed at separate molecular regions. PMID:15063003

  2. Anti-asialo GM1 antiserum treatment of lethally irradiated recipients before bone marrow transplantation: Evidence that recipient natural killer depletion enhances survival, engraftment, and hematopoietic recovery

    SciTech Connect

    Tiberghien, P.; Longo, D.L.; Wine, J.W.; Alvord, W.G.; Reynolds, C.W. )

    1990-10-01

    Natural killer (NK) cells are reported to have an important role in the resistance of lethally irradiated recipients to bone marrow transplantation (BMT). Therefore, we investigated the effects of recipient NK depletion on survival, chimerism, and hematopoietic reconstitution after lethal irradiation and the transplantation of limiting amounts of T-cell-deficient bone marrow (BM). When administered before BMT, anti-asialo GM1 (ASGM1) antiserum treatment, effective in depleting in vivo NK activity, was associated with a marked increase in survival in 3 of 3 allogeneic combinations (BALB/c into C3H/HeN, C57B1/6, or C3B6F1). This enhanced survival was independent of the susceptibility of each recipient strain to accept BALB/c BM. Moreover, recipient anti-ASGM1 treatment was also effective in increasing survival in recipients of syngeneic BM, suggesting that NK cells can adversely affect engraftment independent of genetically controlled polymorphic cell surface determinants. Analysis of chimerism in surviving animals 2 months post-BMT showed that recipient NK depletion significantly increased the level of donor engraftment when high doses of BM were transplanted. These studies also demonstrated that anti-ASGM1 pretreatment mainly resulted in an increase in extramedullary hematopoiesis in the second and third week after irradiation. Anti-ASGM1 treatment also dramatically accelerated the rate of appearance of donor-derived cells with a higher level of donor-cell engraftment apparent at a time when the differences in survival between NK-depleted and control BMT recipients became significant. Peripheral cell counts were also affected by NK depletion, with significantly enhanced platelet and red blood cell recovery and a moderate increase in granulocyte recovery.

  3. Therapeutic potential of intracerebroventricular replacement of modified human β-hexosaminidase B for GM2 gangliosidosis.

    PubMed

    Matsuoka, Kazuhiko; Tamura, Tomomi; Tsuji, Daisuke; Dohzono, Yukie; Kitakaze, Keisuke; Ohno, Kazuki; Saito, Seiji; Sakuraba, Hitoshi; Itoh, Kohji

    2011-06-01

    To develop a novel enzyme replacement therapy for neurodegenerative Tay-Sachs disease (TSD) and Sandhoff disease (SD), which are caused by deficiency of β-hexosaminidase (Hex) A, we designed a genetically engineered HEXB encoding the chimeric human β-subunit containing partial amino acid sequence of the α-subunit by structure-based homology modeling. We succeeded in producing the modified HexB by a Chinese hamster ovary (CHO) cell line stably expressing the chimeric HEXB, which can degrade artificial anionic substrates and GM2 ganglioside in vitro, and also retain the wild-type (WT) HexB-like thermostability in the presence of plasma. The modified HexB was efficiently incorporated via cation-independent mannose 6-phosphate receptor into fibroblasts derived from Tay-Sachs patients, and reduced the GM2 ganglioside accumulated in the cultured cells. Furthermore, intracerebroventricular administration of the modified HexB to Sandhoff mode mice restored the Hex activity in the brains, and reduced the GM2 ganglioside storage in the parenchyma. These results suggest that the intracerebroventricular enzyme replacement therapy involving the modified HexB should be more effective for Tay-Sachs and Sandhoff than that utilizing the HexA, especially as a low-antigenic enzyme replacement therapy for Tay-Sachs patients who have endogenous WT HexB. PMID:21487393

  4. Real-Time PCR Genotyping Assay for GM2 Gangliosidosis Variant 0 in Toy Poodles and the Mutant Allele Frequency in Japan

    PubMed Central

    RAHMAN, Mohammad Mahbubur; YABUKI, Akira; KOHYAMA, Moeko; MITANI, Sawane; MIZUKAMI, Keijiro; UDDIN, Mohammad Mejbah; CHANG, Hye-Sook; KUSHIDA, Kazuya; KISHIMOTO, Miori; YAMABE, Remi; YAMATO, Osamu

    2013-01-01

    ABSTRACT GM2 gangliosidosis variant 0 (Sandhoff disease, SD) is a fatal, progressive neurodegenerative lysosomal storage disease caused by mutations of the HEXB gene. In canine SD, a pathogenic mutation (c.283delG) of the canine HEXB gene has been identified in toy poodles. In the present study, a TaqMan probe-based real-time PCR genotyping assay was developed and evaluated for rapid and large-scale genotyping and screening for this mutation. Furthermore, a genotyping survey was carried out in a population of toy poodles in Japan to determine the current mutant allele frequency. The real-time PCR assay clearly showed all genotypes of canine SD. The assay was suitable for large-scale survey as well as diagnosis, because of its high throughput and rapidity. The genotyping survey demonstrated a carrier frequency of 0.2%, suggesting that the current mutant allele frequency is low in Japan. However, there may be population stratification in different places, because of the founder effect by some carriers. Therefore, this new assay will be useful for the prevention and control of SD in toy poodles. PMID:24161966

  5. Magnetic resonance imaging pattern recognition in hypomyelinating disorders

    PubMed Central

    Steenweg, Marjan E.; Vanderver, Adeline; Blaser, Susan; Bizzi, Alberto; de Koning, Tom J.; Mancini, Grazia M. S.; van Wieringen, Wessel N.; Barkhof, Frederik; Wolf, Nicole I.

    2010-01-01

    Hypomyelination is observed in the context of a growing number of genetic disorders that share clinical characteristics. The aim of this study was to determine the possible role of magnetic resonance imaging pattern recognition in distinguishing different hypomyelinating disorders, which would facilitate the diagnostic process. Only patients with hypomyelination of known cause were included in this retrospective study. A total of 112 patients with Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, Pelizaeus–Merzbacher-like disease, infantile GM1 and GM2 gangliosidosis, Salla disease and fucosidosis were included. The brain scans were rated using a standard scoring list; the raters were blinded to the diagnoses. Grouping of the patients was based on cluster analysis. Ten clusters of patients with similar magnetic resonance imaging abnormalities were identified. The most important discriminating items were early cerebellar atrophy, homogeneity of the white matter signal on T2-weighted images, abnormal signal intensity of the basal ganglia, signal abnormalities in the pons and additional T2 lesions in the deep white matter. Eight clusters each represented mainly a single disorder (i.e. Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, infantile GM1 and GM2 gangliosidosis, Pelizaeus–Merzbacher-like disease and fucosidosis); only two clusters contained multiple diseases. Pelizaeus–Merzbacher-like disease was divided between two clusters and Salla disease did not cluster at all. This study shows that it is possible to separate patients with hypomyelination disorders of known cause in clusters based on magnetic resonance imaging abnormalities alone. In most cases of Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, Pelizaeus–Merzbacher-like disease, infantile GM1 and GM2 gangliosidosis and fucosidosis, the imaging pattern gives clues for the diagnosis. PMID:20881161

  6. Recurrent and novel GLB1 mutations in India.

    PubMed

    Bidchol, Abdul Mueed; Dalal, Ashwin; Trivedi, Rakesh; Shukla, Anju; Nampoothiri, Sheela; Sankar, V H; Danda, Sumita; Gupta, Neerja; Kabra, Madhulika; Hebbar, Shrikiran A; Bhat, Ramesh Y; Matta, Divya; Ekbote, Alka V; Puri, Ratna Dua; Phadke, Shubha R; Gowrishankar, Kalpana; Aggarwal, Shagun; Ranganath, Prajnya; Sharda, Sheetal; Kamate, Mahesh; Datar, Chaitanya A; Bhat, Kamalakshi; Kamath, Nutan; Shah, Hitesh; Krishna, Shuba; Gopinath, Puthiya Mundyat; Verma, Ishwar C; Nagarajaram, H A; Satyamoorthy, Kapaettu; Girisha, Katta Mohan

    2015-08-10

    GM1 gangliosidosis is a lysosomal storage disorder caused by mutations in the GLB1 gene, leading to the deficiency of the enzyme β-d-galactosidase. In this study, we report molecular findings in 50 Asian Indian families with GM1 gangliosidosis. We sequenced all the exons and flanking intronic sequences of GLB1 gene. We identified 33 different mutations (20 novel and 13 previously reported). The novel mutations include 12 missense (p.M1?, p.E129Q, p.G134R, p.L236P, p.G262E, p.L297F, p.Y331C, p.G414V, p.K493N, p.L514P, p.P597L, p.T600I), four splicing (c.246-2A>G, c.397-2A>G, c.552+1G>T, c.956-2A>G), three indels (p.R22Qfs*8, p.L24Cfs*47, p.I489Qfs*4) and one nonsense mutation (p.Q452*). Most common mutations identified in this study were c.75+2InsT (14%) and p.L337P (10%). Known mutations accounted for 67% of allele frequency in our cohort of patients, suggesting that these mutations in GLB1 are recurrent across different populations. Twenty three mutations were localized in the TIM barrel domain, β-domain 1 and β-domain 2. In silico sequence and structure analysis of GLB1 reveal that all the novel mutations affect the function and structure of the protein. We hereby report on the largest series of patients with GM1 gangliosidosis and the first from India. PMID:25936995

  7. I-cell disease. A case report and review of the literature.

    PubMed

    Güngör, N; Coşkun, T; Akçören, Z; Cağlar, M

    1994-01-01

    A four-month-old female infant having developmental delay, coarse facial features and dysostosis multiplex is reported with a special emphasis on the differential diagnosis among I-cell disease (ICD). Hurler syndrome and GM1 gangliosidosis. The lysosomal enzyme studies in cultured skin fibroblasts and serum sample of the patient certified the diagnosis of ICD. Foamy cell infiltration of some organs, including the lungs, and microgyria formation were also noted. Genetic counselling was provided and prenatal diagnosis was offered to the couple to detect ICD in the next pregnancy. PMID:8016916

  8. Liquid chromatography/electrospray ionisation-tandem mass spectrometry quantification of GM2 gangliosides in human peripheral cells and plasma.

    PubMed

    Fuller, Maria; Duplock, Stephen; Hein, Leanne K; Rigat, Brigitte A; Mahuran, Don J

    2014-08-01

    GM2 gangliosidosis is a group of inherited neurodegenerative disorders resulting primarily from the excessive accumulation of GM2 gangliosides (GM2) in neuronal cells. As biomarkers for categorising patients and monitoring the effectiveness of developing therapies are lacking for this group of disorders, we sought to develop methodology to quantify GM2 levels in more readily attainable patient samples such as plasma, leukocytes, and cultured skin fibroblasts. Following organic extraction, gangliosides were partitioned into the aqueous phase and isolated using C18 solid-phase extraction columns. Relative quantification of three species of GM2 was achieved using LC/ESI-MS/MS with d35GM1 18:1/18:0 as an internal standard. The assay was linear over the biological range, and all GM2 gangliosidosis patients were demarcated from controls by elevated GM2 in cultured skin fibroblast extracts. However, in leukocytes only some molecular species could be used for differentiation and in plasma only one was informative. A reduction in GM2 was easily detected in patient skin fibroblasts after a short treatment with media from normal cells enriched in secreted β-hexosaminidase. This method may show promise for measuring the effectiveness of experimental therapies for GM2 gangliosidosis by allowing quantification of a reduction in the primary storage burden. PMID:24769373

  9. Population analysis of the GLB1 gene in South Brazil

    PubMed Central

    Baiotto, Cléia; Sperb, Fernanda; Matte, Ursula; da Silva, Cláudia Dornelles; Sano, Renata; Coelho, Janice Carneiro; Giugliani, Roberto

    2011-01-01

    Infantile GM1 gangliosidosis is caused by the absence or reduction of lysosomal beta-galactosidase activity. Studies conducted in Brazil have indicated that it is one of the most frequent lysosomal storage disorders in the southern part of the country. To assess the incidence of this disorder, 390 blood donors were tested for the presence of two common mutations (1622–1627insG and R59H) in the GLB1 gene. Another group, consisting of 26 GM1 patients, and the blood donors were tested for the presence of two polymorphisms (R521C and S532G), in an attempt to elucidate whether there is a founder effect. The frequencies of the R59H and 1622–1627insG mutations among the GM1 patients studied were 19.2% and 38.5%, respectively. The frequency of polymorphism S532G was 16.7%, whereas R521C was not found in the patients. The overall frequency of either R59H or 1622–1627insG was 57.7% of the disease-causing alleles. This epidemiological study suggested a carrier frequency of 1:58. Seven different haplotypes were found. The 1622–1627insG mutation was not found to be linked to any polymorphism, whereas linkage disequilibrium was found for haplotype 2 (R59H, S532G) (p < 0.001). These data confirm the high incidence of GM1 gangliosidosis and the high frequency of two common mutations in southern Brazil. PMID:21637542

  10. Structural Basis of Pharmacological Chaperoning for Human β-Galactosidase*

    PubMed Central

    Suzuki, Hironori; Ohto, Umeharu; Higaki, Katsumi; Mena-Barragán, Teresa; Aguilar-Moncayo, Matilde; Ortiz Mellet, Carmen; Nanba, Eiji; Garcia Fernandez, Jose M.; Suzuki, Yoshiyuki; Shimizu, Toshiyuki

    2014-01-01

    GM1 gangliosidosis and Morquio B disease are autosomal recessive diseases caused by the defect in the lysosomal β-galactosidase (β-Gal), frequently related to misfolding and subsequent endoplasmic reticulum-associated degradation. Pharmacological chaperone (PC) therapy is a newly developed molecular therapeutic approach by using small molecule ligands of the mutant enzyme that are able to promote the correct folding and prevent endoplasmic reticulum-associated degradation and promote trafficking to the lysosome. In this report, we describe the enzymological properties of purified recombinant human β-GalWT and two representative mutations in GM1 gangliosidosis Japanese patients, β-GalR201C and β-GalI51T. We have also evaluated the PC effect of two competitive inhibitors of β-Gal. Moreover, we provide a detailed atomic view of the recognition mechanism of these compounds in comparison with two structurally related analogues. All compounds bind to the active site of β-Gal with the sugar-mimicking moiety making hydrogen bonds to active site residues. Moreover, the binding affinity, the enzyme selectivity, and the PC potential are strongly affected by the mono- or bicyclic structure of the core as well as the orientation, nature, and length of the exocyclic substituent. These results provide understanding on the mechanism of action of β-Gal selective chaperoning by newly developed PC compounds. PMID:24737316

  11. Lending a helping hand, screening chemical libraries for compounds that enhance beta-hexosaminidase A activity in GM2 gangliosidosis cells.

    PubMed

    Tropak, Michael B; Mahuran, Don

    2007-10-01

    Enzyme enhancement therapy is an emerging therapeutic approach that has the potential to treat many genetic diseases. Candidate diseases are those associated with a mutant protein that has difficulty folding and/or assembling into active oligomers in the endoplasmic reticulum. Many lysosomal storage diseases are candidates for enzyme enhancement therapy and have the additional advantage of requiring only 5-10% of normal enzyme levels to reduce and/or prevent substrate accumulation. Our long experience in working with the beta-hexosaminidase (EC 3.2.1.52) isozymes system and its associated deficiencies (Tay-Sachs and Sandhoff disease) lead us to search for possible enzyme enhancement therapy-agents that could treat the chronic forms of these diseases which express 2-5% residual activity. Pharmacological chaperones are enzyme enhancement therapy-agents that are competitive inhibitors of the target enzyme. Each of the known beta-hexosaminidase inhibitors (low microm IC50) increased mutant enzyme levels to >or= 10% in chronic Tay-Sachs fibroblasts and also attenuated the thermo-denaturation of beta-hexosaminidase. To expand the repertoire of pharmacological chaperones to more 'drug-like' compounds, we screened the Maybridge library of 50,000 compounds using a real-time assay for noncarbohydrate-based beta-hexosaminidase inhibitors and identified several that functioned as pharmacological chaperones in patient cells. Two of these inhibitors had derivatives that had been tested in humans for other purposes. These observations lead us to screen the NINDS library of 1040 Food and Drug Administration approved compounds for pharmacological chaperones. Pyrimethamine, an antimalarial drug with well documented pharmacokinetics, was confirmed as a beta-hexosaminidase pharmacological chaperone and compared favorably with our best carbohydrate-based pharmacological chaperone in patient cells with various mutant genotypes. PMID:17894780

  12. Chaperone therapy for Krabbe disease: potential for late-onset GALC mutations.

    PubMed

    Hossain, Mohammad Arif; Higaki, Katsumi; Saito, Seiji; Ohno, Kazuki; Sakuraba, Hitoshi; Nanba, Eiji; Suzuki, Yoshiyuki; Ozono, Keiichi; Sakai, Norio

    2015-09-01

    Krabbe disease is an autosomal recessive leukodystrophy caused by a deficiency of the galactocerebrosidase (GALC) enzyme. Hematopoietic stem cells transplantation is the only available treatment option for pre-symptomatic patients. We have previously reported the chaperone effect of N-octyl-4-epi-β-valienamine (NOEV) on mutant GM1 β-galactosidase proteins, and in a murine GM1-gangliosidosis model. In this study, we examined its chaperone effect on mutant GALC proteins. We found that NOEV strongly inhibited GALC activity in cell lysates of GALC-transfected COS1 cells. In vitro NOEV treatment stabilized GALC activity under heat denaturation conditions. We also examined the effect of NOEV on cultured COS1 cells expressing mutant GALC activity and human skin fibroblasts from Krabbe disease patients: NOEV significantly increased the enzyme activity of mutants of late-onset forms. Moreover, we confirmed that NOEV could enhance the maturation of GALC precursor to its mature active form. Model structural analysis showed NOEV binds to the active site of human GALC protein. These results, for the first time, provide clear evidence that NOEV is a chaperone with promising potential for patients with Krabbe disease resulting from the late-onset mutations. PMID:26108143

  13. Sustained normalization of neurological disease after intracranial gene therapy in a feline model**

    PubMed Central

    McCurdy, Victoria J.; Johnson, Aime K.; Gray-Edwards, Heather; Randle, Ashley N.; Brunson, Brandon L.; Morrison, Nancy E.; Salibi, Nouha; Johnson, Jacob A.; Hwang, Misako; Beyers, Ronald J.; Leroy, Stanley G.; Maitland, Stacy; Denney, Thomas S.; Cox, Nancy R.; Baker, Henry J.; Sena-Esteves, Miguel; Martin, Douglas R.

    2015-01-01

    Progressive debilitating neurological defects characterize feline GM1 gangliosidosis, a lysosomal storage disease caused by deficiency of lysosomal β-galactosidase. No effective therapy exists for affected children, who often die before age 5. In the current study, an adeno-associated viral vector carrying the therapeutic gene was injected bilaterally into two brain targets (thalamus and deep cerebellar nuclei) of a feline model of GM1 gangliosidosis. Gene therapy normalized β-galactosidase activity and storage throughout the brain and spinal cord. The mean survival of 12 treated GM1 animals was >38 months compared to 8 months for untreated animals. Seven of the 8 treated animals remaining alive demonstrated normalization of disease, with abrogation of many symptoms including gait deficits and postural imbalance. Sustained correction of the GM1 gangliosidosis disease phenotype after limited intracranial targeting by gene therapy in a large animal model suggests that this approach may be useful for treating the human version of this lysosomal storage disorder. PMID:24718858

  14. A comparative study of cytoplasmic granules imaged by the real-time microscope, Nile Red and Filipin in fibroblasts from patients with lipid storage diseases.

    PubMed

    Pham, N-A; Gal, M R; Bagshaw, R D; Mohr, A J; Chue, B; Richardson, T; Callahan, J W

    2005-01-01

    Cytoplasmic granules in fibroblasts, visualized without stains, or labelled with Nile red, Filipin, or anti-LAMP-1 (lysosome-associated membrane protein 1), were imaged using the real-time microscope (RTM). New advances in light microscope technology were applied to detect cytoplasmic granules (RTM-visible granules) and characterize them by imaging contrast, size, shape, cellular distribution, composition, motion dynamics and quantity. Appearing as solid spheroids or ring structures, the majority of the RTM-visible granules contained Nile-red labelled neutral lipids. A smaller subpopulation, appearing dimmer, with less imaging contrast, contained Filipin-labelled free cholesterol. Most lipid storage granules have a diameter ranging from 0.3 mum to 0.6 mum, with a small population measuring up to 1 mum. They typically clustered in the perinuclear region and displayed relatively small oscillatory motion. Immunofluorescence based on LAMP-1 labelling highlighted granular structures that were distinct and separate from RTM-visible granules and other structures in the light modality of the microscope. RTM-visible granules were associated with disease phenotypes that have increased cellular neutral lipid stores corresponding to the Nile red-labelled droplets (e.g. triacylglycerides, cholesterol esters). As predicted, the fibroblast strains with a defect resulting in Wolman disease, when compared to control samples, consistently had RTM-visible granules, higher in imaging contrast and with larger diameters, that were labelled with Nile red, and also an increased frequency of Filipin-cholesterol complexes. By comparison, in fibroblasts where the lipid storage is less evident (Gaucher and Farber diseases) or from GM(1) gangliosidosis, where the primary storage substances are oligosaccharides, fewer and smaller RTM-visible granules were observed. In some cases, changes in contrast and morphology in the unstained cytoplasmic compartments were more evident than in the labelled structures. In summary, applying the RTM imaging system to fibroblasts enables differences between the various disease types to be seen and, in specific examples, a unique phenotype can be readily discerned. PMID:16435192

  15. Antibodies to Glycoproteins Shared by Human Peripheral Nerve and Campylobacter jejuni in Patients with Multifocal Motor Neuropathy

    PubMed Central

    Poceva-Panovska, Ana; Knežević Apostolski, Sladjana

    2013-01-01

    We have tested serum samples from 24 patients with multifocal motor neuropathy (MMN) for reactivity to ganglioside GM1 and to Gal(β1–3)GalNAc-bearing glycoproteins isolated from human peripheral nerve and from Campylobacter jejuni (Cj) serotype O:19. IgM anti-GM1 antibodies were detected by ELISA in 11 patients (45.8%) with MMN and in only one subject (4%) from the control group. Western blots showed positive reactivity of sera from 6 patients (25%) with MMN to several Gal(β1–3)GalNAc-bearing glycoproteins from human peripheral nerve and from Cj O:19 isolates. Sera from three patients (12.5%) with MMN showed positively reactive bands with similar electrophoretic mobility in all isolates (60–62 kDa, 48–51 kDa, 42 kDa, and 38 kDa). All six patients showed positive reactivity to 48–52 kDa protein isolated from human peripheral nerve. Increased titer of IgG antibodies to 60–62 kDa protein isolated from Cj O:19 associated with Guillain-Barré syndrome was detected in three patients, and their serum showed also IgG positive reactivity to peripheral nerve antigen with the same electrophoretic mobility. One of these patients had a previous history of Cj infection which suggests the possibility that Cj may be also involved in the pathogenesis of MMN. PMID:23762534

  16. Biochemistry and genetics of gangliosidoses.

    PubMed

    Sandhoff, K; Christomanou, H

    1979-01-01

    The gangliosidoses comprise an-ever increasing number of biochemically and phenotypically variant diseases. In most of them an autosomal recessive inherited deficiency of a lysosomal hydrolase results in the fatal accumulation of glucolipids (predominantly in the nervous tissue) and of oligosaccharides. The structure, substrate specificity, immunological properties of and genetic studies on the relevant glycosidases, ganglioside GM1 beta-galactosidase and beta-hexosaminidase isoenzymes, are reviewed in this paper. Contrary to general expectation, only a poor correlation is observed between the severity of the disease and residual activity of the defective enzyme when measured with synthetic or natural substrates in the presence of detergents. For the understanding of variant diseases and for their pre- and postnatal diagnosis, the necessity of studying the substrate specificity of normal and mutated enzymes under conditions similar to the in vivo situation, e.g., with natural substrates in the presence of appropriate activator proteins, is stressed. The possibility that detergents may have adverse affects on the substrate specificity of the enzymes is discussed for the beta-hexosaminidases. The significance of activator proteins for the proper interaction of lipid substrates and water-soluble hydrolases is illustrated by the fatal glycolipid storage resulting from an activator protein deficiency in the AB variant of GM2-gangliosidosis. Recent somatic complementation studies have revealed the existence of a presumably post-translational modification factor necessary for the expression of ganglioside GM1 beta-galactosidase activity. This factor is deficient in a group of variants of GM1-glangliosidosis. Among the possible reasons for the variability of enzyme activity levels in heterozygotes and patients, allelic mutations, formation of hybrid enzymes, and the existence of patients as compound heterozygotes are discussed. All these may result in the production of mutant enzymes with an altered specificity for a variety of natural substrates. PMID:116955

  17. Comparison of the canine and human acid {beta}-galactosidase gene

    SciTech Connect

    Ahern-Rindell, A.J.; Kretz, K.A.; O`Brien, J.S.

    1996-05-17

    Several canine cDNA libraries were screened with human {beta}-galactosidase cDNA as probe. Seven positive clones were isolated and sequenced yielding a partial (2060 bp) canine {beta}-galactosidase cDNA with 86% identity to the human {beta}-galactosidase cDNA. Preliminary analysis of a canine genomic library indicated conservation of exon number and size. Analysis by Northern blotting disclosed a single mRNA of 2.4 kb in fibroblasts and liver from normal dogs and dogs affected with GM1 gangliosidosis. Although incomplete, these results indicate canine GM1 gangliosidosis is a suitable animal model of the human disease and should further efforts to devise a gene therapy strategy for its treatment. 20 refs., 2 figs., 1 tab.

  18. Synthesis of lipophilic 1-deoxygalactonojirimycin derivatives as D-galactosidase inhibitors.

    PubMed

    Schitter, Georg; Scheucher, Elisabeth; Steiner, Andreas J; Stütz, Arnold E; Thonhofer, Martin; Tarling, Chris A; Withers, Stephen G; Wicki, Jacqueline; Fantur, Katrin; Paschke, Eduard; Mahuran, Don J; Rigat, Brigitte A; Tropak, Michael; Wrodnigg, Tanja M

    2010-01-01

    N-Alkylation at the ring nitrogen of the D-galactosidase inhibitor 1-deoxygalactonojirimycin with a functionalised C ₆alkyl chain followed by modification with different aromatic substituents provided lipophilic 1-deoxygalactonojirimycin derivatives which exhibit inhibitory properties against β-glycosidases from E. coli and Agrobacterium sp. as well as green coffee bean α-galactosidase. In preliminary studies, these compounds also showed potential as chemical chaperones for GM1-gangliosidosis related β-galactosidase mutants. PMID:20502610

  19. A capillary electrophoresis procedure for the screening of oligosaccharidoses and related diseases.

    PubMed

    Casado, Mercedes; Altimira, Laura; Montero, Raquel; Castejón, Esperanza; Nascimento, Andrés; Pérez-Dueñas, Belén; Ormazabal, Aida; Artuch, Rafael

    2014-07-01

    The most widely used method for the biochemical screening of oligosaccharidoses is the analysis of the urinary oligosaccharide pattern by thin-layer chromatography on silica gel plates. However, this method is not always sensitive enough, and it is extremely time-consuming and laborious. In this work, the analysis of the urine oligosaccharide pattern was standardized for the first time by using capillary electrophoresis with laser-induced fluorescence (CE-LIF) detection (Beckman P/ACE MDQ) with a 488-nm argon ion laser module. All of the analyses were conducted using the Carbohydrate Labeling and Analysis Kit (Beckman-Coulter), which derivatizes samples with 8-aminopyrene-1,3,6-trisulfonate. Urine samples from 40 control subjects (age range, 1 week to 16 years) and from ten patients diagnosed with eight different lysosomal diseases (six of them included in the Educational Oligosaccharide Kit from ERNDIM EQA schemes) were analyzed. Two oligosaccharide excretion patterns were established in our control population according to age (younger or older than 1 year of age). Abnormal peaks with slower migration times than the tetrasaccharide position were observed for fucosidosis, α-mannosidosis, GM1 gangliosidosis, GM2 gangliosidosis variant 0, Pompe disease, and glycogen storage disease type 3. In conclusion, the first CE-LIF method to screen for oligosaccharidoses and related diseases, which also present oligosacchariduria, has been standardized. In all of the cases, the urine oligosaccharide analysis was strongly informative and showed abnormal patterns that were not present in any of the urine samples from the control subjects. Only urine from patients with aspartylglucosaminuria and Schindler disease displayed normal results. PMID:24788891

  20. Genetics Home Reference: GM2-gangliosidosis, AB variant

    MedlinePlus

    ... Living with Inherited Metabolic Diseases (CLIMB) (UK) National Organization for Rare Disorders (NORD): Tay Sachs ... This Page Chen B, Rigat B, Curry C, Mahuran DJ. Structure of the GM2A gene: identification of an exon 2 nonsense mutation and a ...

  1. Genetic variants of immunoglobulin γ and κ chains influence humoral immunity to the cancer-testis antigen XAGE-1b (GAGED2a) in patients with non-small cell lung cancer

    PubMed Central

    Pandey, J P; Namboodiri, A M; Ohue, Y; Oka, M; Nakayama, E

    2014-01-01

    GM (γ marker) allotypes, genetic variants of immunoglobulin γ chains, have been reported to be associated strongly with susceptibility to lung cancer, but the mechanism(s) underlying this association is not known. One mechanism could involve their contribution to humoral immunity to lung tumour-associated antigens. In this study, we aimed to determine whether particular GM and KM (κ marker) allotypes were associated with antibody responsiveness to XAGE-1b, a highly immunogenic lung tumour-associated cancer-testis antigen. Sera from 89 patients with non-small cell lung cancer (NSCLC) were allotyped for eight GM and two KM determinants and characterized for antibodies to a synthetic XAGE-1b protein. The distribution of various GM phenotypes was significantly different between XAGE-1b antibody-positive and-negative patients (P = 0·023), as well as in the subgroup of XAGE-1b antigen-positive advanced NSCLC (P = 0·007). None of the patients with the GM 1,17 21 phenotype was positive for the XAGE-1b antibody. In patients with antigen-positive advanced disease, the prevalence of GM 1,2,17 21 was significantly higher in the antibody-positive group than in those who lacked the XAGE-1b antibody (P = 0·026). This phenotype also interacted with a particular KM phenotype: subjects with GM 1,2,17 21 and KM 3,3 phenotypes were almost four times (odds ratio = 3·8) as likely to be positive for the XAGE-1b antibody as the subjects who lacked these phenotypes. This is the first report presenting evidence for the involvement of immunoglobulin allotypes in immunity to a cancer-testis antigen, which has important implications for XAGE-1b-based immunotherapeutic interventions in lung adenocarcinoma. PMID:24304136

  2. Molecular basis of adult-onset and chronic G sub M2 gangliosidoses in patients of Ashkenazi Jewish origin: Substitution of serine for glycine at position 269 of the. alpha. -subunit of. beta. -hexosaminidase

    SciTech Connect

    Paw, B.H.; Kaback, M.M.; Neufeld, E.F. )

    1989-04-01

    Chronic and adult-onset G{sub M2} gangliosidoses are neurological disorders caused by marked deficiency of the A isoenzyme of {beta}-hexosaminidase; they occur in the Ashkenazi Jewish population, though less frequently than classic (infantile) Tay-Sachs disease. Earlier biosynthetic studies had identified a defective {alpha}-subunit that failed to associate with the {beta}-subunit. The authors have now found a guanosine to adenosine transition at the 3{prime} end of exon 7, which causes substitution of serine for glycine at position 269 of the {alpha}-subunit. An RNase protection assay was used to localize the mutation to a segment of mRNA from fibroblasts of a patient with the adult-onset disorder. That segment of mRNA (after reverse transcription) and a corresponding segment of genomic DNA were amplified by the polymerase chain reaction and sequenced by the dideoxy method. The sequence analysis, together with an assay based on the loss of a ScrFI restriction site, showed that the patient was a compound heterozygote who had inherited the 269 (Gly {yields} Ser) mutation from his father and an allelic null mutation from his mother. The 269 (Gly {yields} Ser) mutation, in compound heterozygosity with a presumed null allele, was also found in fetal fibroblasts with an association-defective phenotype and in cells from five patients with chronic G{sub M2} gangliosidosis.

  3. The role of pharmacotherapy in modifying the neurological status of patients with spinal and spinal cord injuries☆

    PubMed Central

    do Vale Ramos, Renato Carlos; Alegrete, Nuno

    2015-01-01

    The aim here was to conduct a review of the literature on pharmacological therapies for modifying the neurological status of patients with spinal cord injuries. The PubMed database was searched for articles with the terms “spinal cord injury AND methylprednisolone/GM1/apoptosis inhibitor/calpain inhibitor/naloxone/tempol/tirilazad”, in Portuguese or in English, published over the last five years. Older studies were included because of their historical importance. The pharmacological groups were divided according to their capacity to interfere with the physiopathological mechanisms of secondary injuries. Use of methylprednisolone needs to be carefully weighed up: other anti-inflammatory agents have shown benefits in humans or in animals. GM1 does not seem to have greater efficacy than methylprednisolone, but longer-term studies are needed. Many inhibitors of apoptosis have shown benefits in in vitro studies or in animals. Naloxone has not shown benefits. Tempol inhibits the main consequences of oxidation at the level of the spinal cord and other antioxidant drugs seem to have an effect superior to that of methylprednisolone. There is an urgent need to find new treatments that improve the neurological status of patients with spinal cord injuries. The benefits from treatment with methylprednisolone have been questioned, with concerns regarding its safety. Other drugs have been studied, and some of these may provide promising alternatives. Additional studies are needed in order to reach conclusions regarding the benefits of these agents in clinical practice. PMID:27218071

  4. Gangliosidoses.

    PubMed

    Patterson, Marc C

    2013-01-01

    The gangliosidoses comprise a family of lysosomal storage diseases characterized by the accumulation of complex glycosphingolipids in the nervous system and other tissues, secondary to the deficient activity of lysosomal hydrolases or their associated activator proteins. GM1 and GM2 gangliosidosis are associated with deficiency of β-galactosidase and β-hexosaminidase respectively. All gangliosidoses are characterized by progressive neurodegeneration, the severity of which is proportional to the residual enzyme activity. The GM1 gangliosidoses are characterized by dysostosis, organomegaly and coarsening in their most severe forms, whereas children with classic infantile GM2 gangliosidosis (Tay-Sachs disease) are usually spared systemic involvement, except in the case of the Sandhoff variant, in which organomegaly may occur. Cherry-red macular spots occur in the early onset forms of the gangliosidoses, but are less frequently seen in the less severe, later onset phenotypes. Macrocephaly, an exaggerated startle response, cognitive decline, seizures, ataxia, and progressive muscular atrophy may occur in different forms of gangliosidosis. The diagnosis is made by assay of enzyme activity, and can be confirmed by mutation analysis. Carrier screening for Tay-Sachs disease has been remarkably successful in reducing the incidence of this disease in the at-risk Ashkenazi population. There are no proven disease-modifying therapies for the gangliosidoses. PMID:23622392

  5. Fast urinary screening of oligosaccharidoses by MALDI-TOF/TOF mass spectrometry

    PubMed Central

    2014-01-01

    Background Oligosaccharidoses, which belong to the lysosomal storage diseases, are inherited metabolic disorders due to the absence or the loss of function of one of the enzymes involved in the catabolic pathway of glycoproteins and indirectly of glycosphingolipids. This enzymatic deficiency typically results in the abnormal accumulation of uncompletely degraded oligosaccharides in the urine. Since the clinical features of many of these disorders are not specific for a single enzyme deficiency, unambiguous screening is critical to limit the number of costly enzyme assays which otherwise must be performed. Methods Here we provide evidence for the advantages of using a MALDI-TOF/TOF (matrix-assisted laser desorption ionization time-of-flight) mass spectrometric (MS) method for screening oligosaccharidoses. Urine samples from previously diagnosed patients or from unaffected subjects were randomly divided into a training set and a blind testing set. Samples were directly analyzed without prior treatment. Results The characteristic MS and MS/MS molecular profiles obtained allowed us to identify fucosidosis, aspartylglucosaminuria, GM1 gangliosidosis, Sandhoff disease, α-mannosidosis, sialidosis and mucolipidoses type II and III. Conclusions This method, which is easily run in less than 30 minutes, is performed in a single step, and is sensitive and specific. Invaluable for clinical chemistry purposes this MALDI-TOF/TOF mass spectrometry procedure is semi-automatizable and suitable for the urinary screening of oligosacharidoses. PMID:24502792

  6. Glycosidases: inborn errors of glycosphingolipid catabolism.

    PubMed

    Ashida, Hisashi; Li, Yu-Teh

    2014-01-01

    Glycosphingolipids (GSLs) are information-rich glycoconjugates that occur in nature mainly as constituents of biomembranes. Each GSL contains a complex carbohydrate chain linked to a ceramide moiety that anchors the molecule to biomembranes. In higher animals, catabolism of GSLs takes place in lysosomes where sugar chains in GSLs are hydrolyzed by exo-glycosidases to cleave a sugar residue from the non-reducing end of a sugar chain. Inborn errors of GSL-catabolism, collectively called sphingolipidoses or GSL-storage diseases, are caused by the deficiency of exo-glycosidases responsible for the degradation of the specific sugar residues at the non-reducing termini in GSLs. This chapter briefly discusses glycone, anomeric, linkage, and aglycone specificities of exo-glycosidases and some of the historical landmarks on their associations with the chemical pathology of the five best known sphingolipidoses: GM1 gangliosidosis, GM2 gangliosidosis (Tay-Sachs disease), Fabry disease, Gaucher disease, and Krabbe disease. PMID:25151392

  7. Antenatal diagnosis of sphingolipid and mucopolysaccharide storage diseases.

    PubMed Central

    Lowden, J. A.; Rudd, N.; Cutz, E.; Doran, T. A.

    1975-01-01

    In 4 years of 24 fetuses at risk for various sphingolipid and mucopolysaccharide storage diseases were examined. Amniocentesis at 16 weeks' gestation was followed in most cases by culture of amniotic fluid cells and measurement in the cells of the activity of the enzyme suspected to be deficient. Six fetuses were affected; five were examined morphologically and biochemically after abortion. Two fetuses had Tay-Sachs disease, two had GM1 gangliosidosis and one had Hurler's syndrome. Although in each affected detus the specific enzyme activity was absent, we found in the placenta 5 to 50% of the normal activity. Images FIG. 1 PMID:808260

  8. Selective screening for lysosomal storage diseases with dried blood spots collected on filter paper in 4,700 high-risk colombian subjects.

    PubMed

    Uribe, Alfredo; Giugliani, Roberto

    2013-01-01

    Lysosomal storage disorders (LSDs) are a very heterogeneous group of hereditary disorders. The diagnostic process usually involves complex sampling, processing, testing, and validation procedures, performed by specialized laboratories only, which causes great limitations in reaching a diagnosis for patients affected by these diseases.There are few studies about LSDs in Colombia. The diagnostic limitations often make medical practitioners disregard the possibility of these disorders while diagnosing their patients. The current study documents the results of a 7-year screening in high-risk patients, aimed to detect LSDs using dried blood spots (DBS) collected on filter paper, with a micromethodology that facilitates diagnosis even with a large number of samples.The activities of α-galactosidase A, α glucosidase, α-L-iduronidase, arylsulfatase B, β-galactosidase, β-glucosidase, total hexosaminidase, iduronate sulfatase, and chitotriosidase were analyzed in high-risk patients for lysosomal disease. The catalytic activity was evaluated with fluorometric micromethods using artificial substrates marked with 4-methylumbelliferone.The reference values for a control population were established for the enzymes listed above, and 242 patients were found to have an enzyme deficiency, guiding to the following diagnoses: Fabry disease (n = 31), Pompe disease (n = 16), Hurler Syndrome (n = 15), Maroteaux-Lamy Syndrome (n = 34), GM1 Gangliosidosis (n = 10), Morquio B (n = 1), Gaucher disease (n = 101), Sandhoff disease (n = 1), Mucolipidosis (n = 2), and Hunter Syndrome (n = 31). In conclusion, this protocol provides a comprehensive diagnostic approach which could be carried out in Colombia and made it available to medical services spread around the country, enabling the identification of a large number of patients affected by LSDs, which could potentially benefit from the therapeutic tools already available for many of these diseases. PMID:23609959

  9. A pilot study of gene testing of genetic bone dysplasia using targeted next-generation sequencing.

    PubMed

    Zhang, Huiwen; Yang, Rui; Wang, Yu; Ye, Jun; Han, Lianshu; Qiu, Wenjuan; Gu, Xuefan

    2015-12-01

    Molecular diagnosis of genetic bone dysplasia is challenging for non-expert. A targeted next-generation sequencing technology was applied to identify the underlying molecular mechanism of bone dysplasia and evaluate the contribution of these genes to patients with bone dysplasia encountered in pediatric endocrinology. A group of unrelated patients (n=82), characterized by short stature, dysmorphology and X-ray abnormalities, of which mucopolysacharidoses, GM1 gangliosidosis, mucolipidosis type II/III and achondroplasia owing to FGFR3 G380R mutation had been excluded, were recruited in this study. Probes were designed to 61 genes selected according to the nosology and classification of genetic skeletal disorders of 2010 by Illumina's online DesignStudio software. DNA was hybridized with probes and then a library was established following the standard Illumina protocols. Amplicon library was sequenced on a MiSeq sequencing system and the data were analyzed by MiSeq Reporter. Mutations of 13 different genes were found in 44 of the 82 patients (54%). Mutations of COL2A1 gene and PHEX gene were found in nine patients, respectively (9/44=20%), followed by COMP gene in 8 (18%), TRPV4 gene in 4 (9%), FBN1 gene in 4 (9%), COL1A1 gene in 3 (6%) and COL11A1, TRAPPC2, MATN3, ARSE, TRPS1, SMARCAL1, ENPP1 gene mutations in one patient each (2% each). In conclusion, mutations of COL2A1, PHEX and COMP gene are common for short stature due to bone dysplasia in outpatient clinics in pediatric endocrinology. Targeted next-generation sequencing is an efficient way to identify the underlying molecular mechanism of genetic bone dysplasia. PMID:26377240

  10. Crystal Structure of Human β-Galactosidase

    PubMed Central

    Ohto, Umeharu; Usui, Kimihito; Ochi, Toshinari; Yuki, Kenjiro; Satow, Yoshinori; Shimizu, Toshiyuki

    2012-01-01

    GM1 gangliosidosis and Morquio B are autosomal recessive lysosomal storage diseases associated with a neurodegenerative disorder or dwarfism and skeletal abnormalities, respectively. These diseases are caused by deficiencies in the lysosomal enzyme β-d-galactosidase (β-Gal), which lead to accumulations of the β-Gal substrates, GM1 ganglioside, and keratan sulfate. β-Gal is an exoglycosidase that catalyzes the hydrolysis of terminal β-linked galactose residues. This study shows the crystal structures of human β-Gal in complex with its catalytic product galactose or with its inhibitor 1-deoxygalactonojirimycin. Human β-Gal is composed of a catalytic TIM barrel domain followed by β-domain 1 and β-domain 2. To gain structural insight into the molecular defects of β-Gal in the above diseases, the disease-causing mutations were mapped onto the three-dimensional structure. Finally, the possible causes of the diseases are discussed. PMID:22128166

  11. A study of the strategic alliance for EMS industry: the application of a hybrid DEA and GM (1, 1) approach.

    PubMed

    Wang, Chia Nan; Nguyen, Nhu Ty; Tran, Thanh Tuyen; Huong, Bui Bich

    2015-01-01

    Choosing a partner is a critical factor for success in international strategic alliances, although criteria for partner selection vary between developed and transitional markets. This study aims to develop effective methods to assist enterprise to measure the firms' operation efficiency, find out the candidate priority under several different inputs and outputs, and forecast the values of those variables in the future. The methodologies are constructed by the concepts of Data Envelopment Analysis (DEA) and grey model (GM). Realistic data in four consecutive years (2009-2012) a total of 20 companies of the Electronic Manufacturing Service (EMS) industry that went public are completely collected. This paper tries to help target company-DMU1-to find the right alliance partners. By our proposed approach, the results show the priority in the recent years. The research study is hopefully of interest to managers who are in manufacturing industry in general and EMS enterprises in particular. PMID:25821859

  12. Murine β-galactosidase stability is not dependent on temperature or protective protein/cathepsin A.

    PubMed

    Lambourne, Melissa D; Potter, Murray A

    2011-12-01

    GM1 gangliosidosis, a neurodegenerative disorder, and Morquio B disease, a skeletal disorder, are lysosomal storage disorders caused by inherited defects in the enzyme β-galactosidase (GLB1; EC 3.1.2.23; MIM #611458). Enzyme replacement therapy (ERT), a standard of care for a number of non-neuronopathic lysosomal storage disorders, is not yet available for GLB1 deficiency. Although functionally active recombinant human and feline GLB1 precursors have been purified, ERT has not yet been demonstrated in GM1 gangliosidosis or Morquio B disease models. A major obstacle to developing effective therapy may be the stability of human GLB1. We show here that mouse GLB1 has greater stability when compared to human GLB1, and that human GLB1 activity is temperature and protective-dependent on protein cathepsin A, while that of mouse GLB1 is not. These findings may impact on the eventual development of ERT for GLB1 deficiency. Despite our attempts to improve the extracellular stability of human GLB1 through sequence modification and the use of chemical chaperone N-butyldeoxygalactonojirimycin, the specific enzyme activity remained well below that of mGLB1. PMID:22001501

  13. Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses.

    PubMed

    Akgoc, Zeynep; Sena-Esteves, Miguel; Martin, Douglas R; Han, Xianlin; d'Azzo, Alessandra; Seyfried, Thomas N

    2015-05-01

    Bis(monoacylglycero)phosphate (BMP) is a negatively charged glycerophospholipid with an unusual sn-1;sn-1' structural configuration. BMP is primarily enriched in endosomal/lysosomal membranes. BMP is thought to play a role in glycosphingolipid degradation and cholesterol transport. Elevated BMP levels have been found in many lysosomal storage diseases (LSDs), suggesting an association with lysosomal storage material. The gangliosidoses are a group of neurodegenerative LSDs involving the accumulation of either GM1 or GM2 gangliosides resulting from inherited deficiencies in β-galactosidase or β-hexosaminidase, respectively. Little information is available on BMP levels in gangliosidosis brain tissue. Our results showed that the content of BMP in brain was significantly greater in humans and in animals (mice, cats, American black bears) with either GM1 or GM2 ganglioside storage diseases, than in brains of normal subjects. The storage of BMP and ganglioside GM2 in brain were reduced similarly following adeno-associated viral-mediated gene therapy in Sandhoff disease mice. We also found that C22:6, C18:0, and C18:1 were the predominant BMP fatty acid species in gangliosidosis brains. The results show that BMP accumulates as a secondary storage material in the brain of a broad range of mammals with gangliosidoses. PMID:25795792

  14. Intermittent horizontal saccade failure ('ocular motor apraxia') in children.

    PubMed Central

    Harris, C M; Shawkat, F; Russell-Eggitt, I; Wilson, J; Taylor, D

    1996-01-01

    BACKGROUND: Ocular motor apraxia (OMA) in childhood is a poorly understood condition involving a failure of horizontal saccades. OMA is thought to be rare but the literature indicates wide clinical associations. OMA is often identified by abnormal head movements, but failure of reflexive quick phases has been reported in all but a few patients. The extent of this oculomotor disorder was examined in a large group of children with diverse clinical backgrounds. METHODS: The degree of quick phase failure during horizontal vestibular and optokinetic nystagmus was measured using DC electro-oculography and video in 74 affected children, aged 17 days to 14 years. RESULTS: All children showed an intermittent failure of nystagmic quick phases, except for total failure in one case. Other visuomotor abnormalities were common including saccadic hypometria (85%), low gain smooth pursuit (70%), neurological nystagmus (28%), strabismus (22%), and vertical abnormalities (11%). Non-ocular abnormalities were common including infantile hypotonia (61%), motor delay (77%), and speech delay (87%). There was a wide range of clinical associations including agenesis of the corpus callosum, Joubert syndrome, Dandy-Walker malformation, microcephaly, hydrocephalus, vermis hypoplasia, porencephalic cyst, megalocephaly, Krabbe leucodystrophy, Pelizaeus Merzbacher disease, infantile Gaucher disease, GM1 gangliosidosis, infantile Refsum's disease, propionic acidaemia, ataxia telangiectasia, Bardet-Biedl syndrome, vermis astrocytoma, vermis cyst, carotid fibromuscular hypoplasia, Cornelia de Lange syndrome, and microphthalmos. Perinatal and postnatal problems were found in 15% including perinatal hypoxia, meningitis, periventricular leucomalacia, athetoid cerebral palsy, perinatal septicaemia and anaemia, herpes encephalitis, and epilepsy. Only 27% were idiopathic. CONCLUSION: Quick phase failure is a constant feature of OMA, whereas abnormal head movements were detected in only about half, depending on the underlying diagnosis. This oculomotor sign is better described as an intermittent saccade failure rather than as a true apraxia. It indicates central nervous system involvement, has wide clinical associations, but it is not a diagnosis. PMID:8814747

  15. Glucosylceramide modulates endolysosomal pH in Gaucher disease.

    PubMed

    Sillence, Dan J

    2013-06-01

    GlcCer accumulation causes Gaucher disease where GlcCer breakdown is inhibited due to a hereditary deficiency in glucocerebrosidase. Glycolipids are endocytosed and targeted to the Golgi apparatus in normal cells but in Gaucher disease they are mistargeted to lysosomes. To better understand the role of GlcCer in endocytic sorting RAW macrophages were treated with Conduritol B-epoxide to inhibit GlcCer breakdown. Lipid analysis found increases in GlcCer led to accumulation of both triacylglycerol and cholesterol consistent with increased lysosomal pH. Ratio imaging of macrophages using both acridine orange and lysosensor yellow/blue to measure endolysosomal pH revealed increases in Conduritol B-epoxide treated RAW macrophages and Gaucher patient lymphoblasts. Increased endolysosomal pH was restricted to Gaucher lymphoblasts as no significant increases in pH were seen in Fabry, Krabbe, Tay-Sachs and GM1-gangliosidosis lymphoblasts. Substrate reduction therapy utilises inhibitors of GlcCer synthase to reduce storage in Gaucher disease. The addition of inhibitors of GlcCer synthesis to RAW macrophages also led to increases in cholesterol and triacylglycerol and an endolysosomal pH increase of up to 1 pH unit. GlcCer modulation appears specific since glucosylsphingosine but not galactosylsphingosine reversed the effects of GlcCer depletion. Although no acute effects on glycolipid trafficking were observed using bafilomycin A the results are consistent with a multistep model whereby increases in pH lead to altered trafficking via cholesterol accumulation. GlcCer modulates endolysosomal pH in lymphocytes suggesting an important role in normal lysosomes which may be disrupted in Gaucher disease. PMID:23628459

  16. Patient Rights

    MedlinePlus

    As a patient, you have certain rights. Some are guaranteed by federal law, such as the right to get a copy ... them private. Many states have additional laws protecting patients, and healthcare facilities often have a patient bill ...

  17. Patient Satisfaction

    PubMed Central

    Prakash, Bhanu

    2010-01-01

    Patient satisfaction is an important and commonly used indicator for measuring the quality in health care. Patient satisfaction affects clinical outcomes, patient retention, and medical malpractice claims. It affects the timely, efficient, and patient-centered delivery of quality health care. Patient satisfaction is thus a proxy but a very effective indicator to measure the success of doctors and hospitals. This article discusses as to how to ensure patient satisfaction in dermatological practice. PMID:21430827

  18. Patient satisfaction.

    PubMed

    Prakash, Bhanu

    2010-09-01

    Patient satisfaction is an important and commonly used indicator for measuring the quality in health care. Patient satisfaction affects clinical outcomes, patient retention, and medical malpractice claims. It affects the timely, efficient, and patient-centered delivery of quality health care. Patient satisfaction is thus a proxy but a very effective indicator to measure the success of doctors and hospitals. This article discusses as to how to ensure patient satisfaction in dermatological practice. PMID:21430827

  19. Gene transfer corrects acute GM2 gangliosidosis--potential therapeutic contribution of perivascular enzyme flow.

    PubMed

    Cachón-González, M Begoña; Wang, Susan Z; McNair, Rosamund; Bradley, Josephine; Lunn, David; Ziegler, Robin; Cheng, Seng H; Cox, Timothy M

    2012-08-01

    The GM2 gangliosidoses are fatal lysosomal storage diseases principally affecting the brain. Absence of β-hexosaminidase A and B activities in the Sandhoff mouse causes neurological dysfunction and recapitulates the acute Tay-Sachs (TSD) and Sandhoff diseases (SD) in infants. Intracranial coinjection of recombinant adeno-associated viral vectors (rAAV), serotype 2/1, expressing human β-hexosaminidase α (HEXA) and β (HEXB) subunits into 1-month-old Sandhoff mice gave unprecedented survival to 2 years and prevented disease throughout the brain and spinal cord. Classical manifestations of disease, including spasticity-as opposed to tremor-ataxia-were resolved by localized gene transfer to the striatum or cerebellum, respectively. Abundant biosynthesis of β-hexosaminidase isozymes and their global distribution via axonal, perivascular, and cerebrospinal fluid (CSF) spaces, as well as diffusion, account for the sustained phenotypic rescue-long-term protein expression by transduced brain parenchyma, choroid plexus epithelium, and dorsal root ganglia neurons supplies the corrective enzyme. Prolonged survival permitted expression of cryptic disease in organs not accessed by intracranial vector delivery. We contend that infusion of rAAV into CSF space and intraparenchymal administration by convection-enhanced delivery at a few strategic sites will optimally treat neurodegeneration in many diseases affecting the nervous system. PMID:22453766

  20. Lipopolysaccharides from Campylobacter jejuni associated with Guillain-Barré syndrome patients mimic human gangliosides in structure.

    PubMed Central

    Aspinall, G O; Fujimoto, S; McDonald, A G; Pang, H; Kurjanczyk, L A; Penner, J L

    1994-01-01

    Lipopolysaccharides extracted from Campylobacter jejuni serostrains (serotype reference strains) for serotypes O:4 and O:19 were found to have core oligosaccharides with terminal structures resembling human gangliosides GM1 and GD1a. High-molecular-weight molecules that reflected the presence of O chains were shown in immunoblots to be immunologically specific for each serostrain. The O:19 antiserum also reacted strongly with core oligosaccharides of two isolates from patients with Guillain-Barré syndrome (GBS), but the banding patterns and molecular structures were different from those of the O:19 serostrain. A neuraminobiose disaccharide unit is attached to the terminal Gal residue in one isolate, and the other isolate lacked terminal N-acetyl glucosamine and galactose with attached sialic acid so that the sialic acid residues were present in a neuraminobiose unit linked to the only remaining galactose. Analysis of the high-M(r) lipopolysaccharides of the O:19 serostrain and the two isolates from GBS patients revealed the presence of a hyaluronic acid-like polymer with disaccharide-repeating units consisting of beta-D-glucuronic acid amidated with 2-amino-2-deoxyglycerol and N-acetyl glucosamine. The results confirm a potential role for the core oligosaccharides in the etiology of GBS but also suggest that the O-chain polysaccharide may be a contributing factor. Images PMID:8168981

  1. BEN Patient

    Balkan endemic nephropathy (BEN) patient from a BEN village in Romania. The photo was taken at a dialysis clinic in Romania where the patient traveled every 2 to 3 days to receive dialysis, the principal treatment option for people with BEN. This patient died from complications of BEN within a year ...

  2. Carrier Rates of Four Single-Gene Disorders in Croatian Bayash Roma

    PubMed Central

    Barešić, Ana

    2014-01-01

    To assess how specific population history, different migration routes, isolation, and endogamy practices contributed to the distribution of several rare diseases found in specific Roma groups, we conducted a population-based research study of rare disease mutations in Croatian Vlax Roma. We tested a total of 427 subjects from Baranja and Međimurje for the presence of four mutations causing hereditary motor and sensory neuropathy type Lom (HMSNL), GM1 gangliosidosis (GM1), congenital cataracts, facial dysmorphism and neuropathy (CCFDN), and limb girdle muscle dystrophy type 2C (LGMD2C), using the RFLP-PCR method to estimate carrier frequencies. We identified a total of four individuals heterozygous for the mutation causing HMSNL in the Baranja population, with a carrier rate amounting to 1.5%. Carriers for other three mutations causing GM1, CCFDN, and LGMD2C were not found in our sample. The carrier rate for the HMSNL mutation in Baranja is lower than in other Vlax Roma groups. In addition, distinct differences in carrier rates between the Croatian Vlax groups point to different genetic history, despite their belonging to the same Roma migration category and subgroup. The difference in carrier rates is either the result of admixture or the reflection of a greater extent of genetic drift since recent founding, maintained by a high degree of endogamy. PMID:24180318

  3. State of the Art: Why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection?

    PubMed Central

    Chmiel, James F; Davis, Pamela B

    2003-01-01

    Cystic Fibrosis (CF) lung disease, which is characterized by airway obstruction, chronic bacterial infection, and an excessive inflammatory response, is responsible for most of the morbidity and mortality. Early in life, CF patients become infected with a limited spectrum of bacteria, especially P. aeruginosa. New data now indicate that decreased depth of periciliary fluid and abnormal hydration of mucus, which impede mucociliary clearance, contribute to initial infection. Diminished production of the antibacterial molecule nitric oxide, increased bacterial binding sites (e.g., asialo GM-1) on CF airway epithelial cells, and adaptations made by the bacteria to the airway microenvironment, including the production of virulence factors and the ability to organize into a biofilm, contribute to susceptibility to initial bacterial infection. Once the patient is infected, an overzealous inflammatory response in the CF lung likely contributes to the host's inability to eradicate infection. In response to increased IL-8 and leukotriene B4 production, neutrophils infiltrate the lung where they release mediators, such as elastase, that further inhibit host defenses, cripple opsonophagocytosis, impair mucociliary clearance, and damage airway wall architecture. The combination of these events favors the persistence of bacteria in the airway. Until a cure is discovered, further investigations into therapies that relieve obstruction, control infection, and attenuate inflammation offer the best hope of limiting damage to host tissues and prolonging survival. PMID:14511398

  4. State of the art: why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection?

    PubMed

    Chmiel, James F; Davis, Pamela B

    2003-01-01

    Cystic Fibrosis (CF) lung disease, which is characterized by airway obstruction, chronic bacterial infection, and an excessive inflammatory response, is responsible for most of the morbidity and mortality. Early in life, CF patients become infected with a limited spectrum of bacteria, especially P. aeruginosa. New data now indicate that decreased depth of periciliary fluid and abnormal hydration of mucus, which impede mucociliary clearance, contribute to initial infection. Diminished production of the antibacterial molecule nitric oxide, increased bacterial binding sites (e.g., asialo GM-1) on CF airway epithelial cells, and adaptations made by the bacteria to the airway microenvironment, including the production of virulence factors and the ability to organize into a biofilm, contribute to susceptibility to initial bacterial infection. Once the patient is infected, an overzealous inflammatory response in the CF lung likely contributes to the host's inability to eradicate infection. In response to increased IL-8 and leukotriene B4 production, neutrophils infiltrate the lung where they release mediators, such as elastase, that further inhibit host defenses, cripple opsonophagocytosis, impair mucociliary clearance, and damage airway wall architecture. The combination of these events favors the persistence of bacteria in the airway. Until a cure is discovered, further investigations into therapies that relieve obstruction, control infection, and attenuate inflammation offer the best hope of limiting damage to host tissues and prolonging survival. PMID:14511398

  5. Patient Injuries?

    PubMed

    2015-01-01

    An injured patient may be the last thing dentists want to think about. However, in reality, patients can be injured during dental treatment or as the result of an incident such as a slip and fall in the office. Treatment-related injuries can run the gamut and include burns, lacerations, swallowed objects and allergic reactions, according to The Dentists Insurance Company. PMID:26638304

  6. Galactonojirimycin derivatives restore mutant human beta-galactosidase activities expressed in fibroblasts from enzyme-deficient knockout mouse.

    PubMed

    Tominaga, L; Ogawa, Y; Taniguchi, M; Ohno, K; Matsuda, J; Oshima, A; Suzuki, Y; Nanba, E

    2001-08-01

    Ten low molecular compounds analogous to galactose were screened for inhibition of human beta-galactosidase activity. Among them, 1-deoxy-galactonojirimycin and N-(n-butyl)-deoxy-galactonojirimycin showed an inhibitory effect at high concentrations. However, they restored mutant enzyme activities expressed in enzyme-deficient knockout mouse fibroblasts and human beta-galactosidosis fibroblasts at lower intracellular concentrations. This effect was more remarkable on G(M1)-gangliosidosis mutations (R201C, I51T, R201H, R457Q) than Morquio B disease mutations (W273L, Y83H). These low molecular compounds pass though the blood-brain barrier in mice. We hope that this new therapeutic approach will become clinically applicable in the near future. PMID:11504597

  7. Extensive Mongolian spots: a clinical sign merits special attention.

    PubMed

    Ashrafi, Mahmood Reza; Shabanian, Reza; Mohammadi, Mahmood; Kavusi, Susan

    2006-02-01

    Although typical and limited Mongolian spots are benign skin markings at birth which fade and disappear as the child grows, extensive Mongolian spots deserve special attention as possible indications of associated inborn error of metabolism. A few cases of extensive Mongolian spots in association with inheritable storage diseases have been reported. Some hypotheses have been put forward, but further investigation is necessary to elucidate the causative factors. This report describes three infants with generalized Mongolian spots, two infants with GM1 gangliosidosis type 1, and one in association with Hurler syndrome. Findings of generalized Mongolian spots in newborns may lead to an early detection and early treatment before irreversible organ damage occurs. PMID:16458829

  8. Fluorous Iminoalditols: A New Family of Glycosidase Inhibitors and Pharmacological Chaperones

    PubMed Central

    Schitter, Georg; Steiner, Andreas J.; Pototschnig, Gerit; Scheucher, Elisabeth; Thonhofer, Martin; Tarling, Chris A.; Withers, Stephen G.; Fantur, Katrin; Paschke, Eduard; Mahuran, Don J.; Rigat, Brigitte A.; Tropak, Michael B.; Illaszewicz, Carina; Saf, Robert; Wrodnigg, Tanja M.

    2011-01-01

    A collection of new reversible glycosidase inhibitors of the iminoalditol type featuring N-substituents containing perfluorinated regions has been prepared for evaluation of physicochemical, biochemical and diagnostic properties. The vast variety of feasible oligofluoro moieties allows for modular approaches to customised structures according to the intended applications, which are influenced by the fluorine content as well as the distance of the fluorous moiety from the ring nitrogen. The first examples, in particular in the D-galacto series, exhibited excellent inhibitory activities. A preliminary screen with two human cell lines showed that, at subinhibitory concentrations, they are powerful pharmacological chaperones enhancing the activities of the catalytically handicapped lysosomal D-galactosidase mutants associated with GM1 gangliosidosis and Morquio B disease. PMID:20715263

  9. A Study of the Strategic Alliance for EMS Industry: The Application of a Hybrid DEA and GM (1, 1) Approach

    PubMed Central

    Wang, Chia Nan; Tran, Thanh Tuyen; Huong, Bui Bich

    2015-01-01

    Choosing a partner is a critical factor for success in international strategic alliances, although criteria for partner selection vary between developed and transitional markets. This study aims to develop effective methods to assist enterprise to measure the firms' operation efficiency, find out the candidate priority under several different inputs and outputs, and forecast the values of those variables in the future. The methodologies are constructed by the concepts of Data Envelopment Analysis (DEA) and grey model (GM). Realistic data in four consecutive years (2009–2012) a total of 20 companies of the Electronic Manufacturing Service (EMS) industry that went public are completely collected. This paper tries to help target company—DMU1—to find the right alliance partners. By our proposed approach, the results show the priority in the recent years. The research study is hopefully of interest to managers who are in manufacturing industry in general and EMS enterprises in particular. PMID:25821859

  10. Patient Monitoring

    NASA Technical Reports Server (NTRS)

    1978-01-01

    In photo above, the electrocardiogram of a hospitalized patient is being transmitted by telemetry. Widely employed in space operations, telemetry is a process wherein instrument data is converted to electrical signals and sent to a receiver where the signals are reconverted to usable information. In this instance, heart readings are picked up by the electrode attached to the patient's body and delivered by wire to the small box shown, which is a telemetry transmitter. The signals are relayed wirelessly to the console in the background, which converts them to EKG data. The data is displayed visually and recorded on a printout; at the same time, it is transmitted to a central control station (upper photo) where a nurse can monitor the condition of several patients simultaneously. The Patient Monitoring System was developed by SCI Systems, Inc., Huntsville, Alabama, in conjunction with Abbott Medical Electronics, Houston, Texas. In developing the system, SCI drew upon its extensive experience as a NASA contractor. The company applied telemetry technology developed for the Saturn launch vehicle and the Apollo spacecraft; instrumentation technology developed for heart, blood pressure and sleep monitoring of astronauts aboard NASA's Skylab long duration space station; and communications technology developed for the Space Shuttle.

  11. Patient Safety

    MedlinePlus

    You can help prevent medical errors by being an active member of your health care team. Research shows that patients who are more involved with their ... get better results. To reduce the risk of medical errors, you can Ask questions if you have doubts ...

  12. Cerebrospinal Fluid Monoamine Metabolite Analysis in Pediatric Movement Disorders.

    PubMed

    Tonduti, Davide; Zorzi, Giovanna; Ghezzi, Daniele; Zibordi, Federica; Garavaglia, Barbara; Nardocci, Nardo

    2015-11-01

    Abnormal concentrations of dopamine and serotonin metabolites in the cerebrospinal fluid is the diagnostic hallmark of a group of treatable conditions known as the monoamine neurotransmitter disorders. We assessed cerebrospinal fluid dopamine and serotonin metabolite concentrations in a series of 69 patients affected by movement disorders of unknown etiology. Abnormal results were disclosed in 13/69 subjects (19%). Both primary and secondary monoamine neurotransmitter disorders were observed. The clinical presentation of both forms was hypokinetic-rigid syndrome or dystonia. L-Dopa treatment resulted in significant improvement of the clinical picture in the majority of primary neurotransmitter disorders. Eight patients presented a secondary neurotransmitter disorder. One suffered from a GM2 gangliosidosis and one from infantile bilateral striatal necrosis. Etiologic diagnoses were not established in the others. L-Dopa was started in four patients, leading to a significant improvement of hypokinesia in the patient suffering from GM2 gangliosidosis and a slight improvement in 3 unclassified patients. PMID:25907776

  13. Patient identification.

    PubMed

    Thorpy, Michael J; Lieberman, Joseph A; Roth, Thomas; Owens, Glenda S

    2007-11-01

    The homeostatic sleep drive and circadian arousal, each opposing the other, form the neurobiological bases of the sleep and wake states. Many factors can and do disrupt this cycle. Yet, excessive daytime sleepiness is not only common, and it often goes unrecognized. It can contribute to accidents, produce or exacerbate health conditions, reduce efficiency and productivity, interfere with social relationships, and diminish quality of life. The spectrum of common sleep disorders includes circadian rhythm changes, shiftwork requirements, obstructive sleep apnea, narcolepsy, and difficulty in initiating or maintaining sleep, also known as insomnia. In many cases, however, sleep deprivation is the choice of the patient, chosen in response to long commutes, academic rigor, or occupational matters. Regardless of the sleep disorder that a patient has, good sleep behavior or "sleep hygiene" is essential. PMID:18041874

  14. Patient's perspective.

    PubMed

    Raffa, Robert B; Martin, Kathleen J

    2010-01-01

    An unknown, but significant subgroup (perhaps the majority), of patients who have undergone chemotherapy treatment for their cancer report a subsequent decline in cognitive performance (e.g., difficulty in balancing a checkbook; forgetting or mixing up names of friends or relatives, etc.). The condition has been termed chemo fog, chemo brain, or some similar term to reflect the fact that the symptoms are usually difficult to describe and involve domains of cognition such as attention, concentration, memory, speed of information processing, multitasking, or ability to organize information. The deficits are reported to persist. The magnitude of the negative impact on quality of life depends, as does the condition itself, on multiple and varied factors. This chapter relates the experience of one patient. PMID:20738000

  15. NORD's Patient Assistance Programs

    MedlinePlus

    ... donate Menu for Patients and Families Information & Resources Rare Disease Information Resources & FAQs State Health Insurance Information Information ... Find a Patient Organization Find a Patient Meeting Rare Disease Day Start a Patient Organization Patient Stories Take ...

  16. Effect of Extra Virgin Olive Oil on Biomarkers of Inflammation in HIV-Infected Patients: A Randomized, Crossover, Controlled Clinical Trial

    PubMed Central

    Dokmanović, Sanja Kozić; Kolovrat, Krunoslava; Laškaj, Renata; Jukić, Vedrana; Vrkić, Nada; Begovac, Josip

    2015-01-01

    Background Premature atherosclerosis in HIV-infected patients is associated with chronic infection by itself and adverse effects of antiretroviral treatment (ART). Extra virgin olive oil (EVOO) has a beneficial effect on the cardiovascular system because of its anti-inflammatory properties. The objective of this study was to determine whether the consumption of EVOO improves inflammation and atherosclerosis biomarkers in HIV-infected patients receiving ART. Material/Methods This randomized, crossover, controlled trial included 39 HIV-positive male participants who consumed 50 mL of EVOO or refined olive oil (ROO) daily. Four participants dropped out of the study. Leukocyte count, erythrocyte sedimentation rate (ESR), high-sensitivity C-reactive protein (hsCRP), interleukin-6, fibrinogen, total cholesterol, LDL cholesterol, HDL cholesterol, triglycerides, malondialdehyde, glutathione-peroxidase, superoxide dismutase, oxidized LDL and von Willebrand factor were determined before the first and after each of the 2 intervention periods. Intervention and washout periods lasted for 20 and 14 days, respectively. Results In participants with >90% compliance (N=30), hsCRP concentrations were lower after EVOO intervention (geometric mean [GM], 1.70 mg/L; 95% confidence interval [CI], 1.15–2.52) compared to ROO administration (GM, 2.92 mg/L; 95% CI, 1.95–4.37) (p=0.035). In participants using lopinavir/ritonavir, ESR and hsCRP concentrations decreased 62% and 151%, respectively, after EVOO administration. In the whole study population (N=35) we found no difference in analyzed biomarkers after EVOO administration. Conclusions Our exploratory study suggests that EVOO consumption could lower hsCRP in patients on ART. PMID:26280823

  17. Increased catabolism and decreased unsaturation of ganglioside in patients with inflammatory bowel disease

    PubMed Central

    Miklavcic, John J; Hart, Tasha DL; Lees, Gordon M; Shoemaker, Glen K; Schnabl, Kareena L; Larsen, Bodil MK; Bathe, Oliver F; Thomson, Alan BR; Mazurak, Vera C; Clandinin, M Tom

    2015-01-01

    AIM: To investigate whether accelerated catabolism of ganglioside and decreased ganglioside content contribute to the etiology of pro-inflammatory intestinal disease. METHODS: Intestinal mucosa from terminal ileum or colon was obtained from patients with ulcerative colitis or inflammatory Crohn’s disease (n = 11) undergoing bowel resection and compared to control samples of normal intestine from patients with benign colon polyps (n = 6) and colorectal cancer (n = 12) in this observational case-control study. Gangliosides and phospholipids of intestinal mucosa were characterized by class and ceramide or fatty acid composition using liquid chromatography triple-quad mass spectrometry. Content and composition of ganglioside classes GM1, GM3, GD3, GD1a, GT1 and GT3 were compared among subject groups. Content and composition of phospholipid classes phosphatidylcholine (PC) and phosphatidylethanolamine were compared among subject groups. Unsaturation index of individual ganglioside and phospholipid classes was computed and compared among subject groups. Ganglioside catabolism enzymes beta-hexosaminidase A (HEXA) and sialidase-3 (NEU3) were measured in intestinal mucosa using western blot and compared among subject groups. RESULTS: Relative GM3 ganglioside content was 2-fold higher (P < 0.05) in intestine from patients with inflammatory bowel disease (IBD) compared to control intestine. The quantity of GM3 and ratio of GM3/GD3 was also higher in IBD intestine than control tissue (P < 0.05). Control intestine exhibited 3-fold higher (P < 0.01) relative GD1a ganglioside content than IBD intestine. GD3 and GD1a species of ganglioside containing three unsaturated bonds were present in control intestine, but were not detected in IBD intestine. The relative content of PC containing more than two unsaturated bonds was 30% lower in IBD intestine than control intestine (P < 0.05). The relative content of HEXA in IBD intestine was increased 1.7-fold (P < 0.05) and NEU3 was increased 8.3-fold (P < 0.01) compared to normal intestine. Intestinal mucosa in IBD is characterized by increased GM3 content, decreased GD1a, and a reduction in polyunsaturated fatty acid constituents in GD3, GD1a and PC. CONCLUSION: This study suggests a new paradigm by proposing that IBD occurs as a consequence of increased metabolism of specific gangliosides. PMID:26401073

  18. Neural precursor cell cultures from GM2 gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology.

    PubMed

    Martino, Sabata; di Girolamo, Ilaria; Cavazzin, Chiara; Tiribuzi, Roberto; Galli, Rossella; Rivaroli, Anna; Valsecchi, Manuela; Sandhoff, Konrad; Sonnino, Sandro; Vescovi, Angelo; Gritti, Angela; Orlacchio, Aldo

    2009-04-01

    In this work we showed that genotype-related patterns of hexosaminidase activity, isoenzyme composition, gene expression and ganglioside metabolism observed during embryonic and postnatal brain development are recapitulated during the progressive stages of neural precursor cell (NPC) differentiation to mature glia and neurons in vitro. Further, by comparing NPCs and their differentiated progeny established from Tay-Sachs (TS) and Sandhoff (SD) animal models with the wild-type counterparts, we studied the events linking the accumulation of undegraded substrates to hexosaminidase activity. We showed that similarly to what observed in brain tissues in TS NPCs and progeny, the stored GM2 was partially converted by sialidase to GA2, which can be then degraded in the lysosomes to its components. The latter can be used in a salvage pathway for the formation of GM3. Interestingly, results obtained from ganglioside feeding assays and from measurement of lysosomal sialidase activity suggest that a similar pathway might work also in the SD model. PMID:19166507

  19. [Patient advice].

    PubMed

    Lucio-Villegas Menéndez, M Eulalia; González, Laura López; Gutiérrez Pérez, M Isabel; Lluch, Natalia Aresté; Morató Agustí, M Luisa; Cachafeiro, Santiago Pérez

    2014-05-01

    In wound care, knowing what to do is as important as knowing what not to do. The first step is to evaluate the severity of the lesion and to know whether it is necessary to attend a health center or not. If the wound is simple, the recommended course of action is cleansing with serum or water after washing one's hands, followed by wound disinfection with the most appropriate antiseptic. Antiseptics not should be used for wound cleansing (physiological serum or tap water should be used) or for wound healing with granulation tissue. Equally, antiseptics should not be used in the ear or near the eyes; if there is accidental application, the eye should be washed in abundant water. Povidone iodine should not be used in pregnant women, nor should iodine preparations be used in neonates, in patients with thyroid alterations or in those allergic to iodine. Currently, merbromine/mercurochrome is not used because of its mercury content. Before an antiseptic is applied, all inorganic residues (foreign bodies) and dead tissue should be removed; detritus, slough, purulent exudate, scabs… This will aid healing and the action of antiseptics, since they become inactive in the presence of organic material. PMID:24998084

  20. The prevalence of nine genetic disorders in a dog population from Belgium, the Netherlands and Germany.

    PubMed

    Broeckx, Bart J G; Coopman, Frank; Verhoeven, Geert E C; Van Haeringen, Wim; van de Goor, Leanne; Bosmans, Tim; Gielen, Ingrid; Saunders, Jimmy H; Soetaert, Sandra S A; Van Bree, Henri; Van Neste, Christophe; Van Nieuwerburgh, Filip; Van Ryssen, Bernadette; Verelst, Elien; Van Steendam, Katleen; Deforce, Dieter

    2013-01-01

    The objective of this study was to screen a dog population from Belgium, the Netherlands and Germany for the presence of mutant alleles associated with hip dysplasia (HD), degenerative myelopathy (DM), exercise-induced collapse (EIC), neuronal ceroid lipofuscinosis 4A (NCL), centronuclear myopathy (HMLR), mucopolysaccharidosis VII (MPS VII), myotonia congenita (MG), gangliosidosis (GM1) and muscular dystrophy (Duchenne type) (GRMD). Blood samples (K3EDTA) were collected for genotyping with Kompetitive Allele Specific PCR (n = 476). Allele and genotype frequencies were calculated in those breeds with at least 12 samples (n = 8). Hardy-Weinberg equilibrium was tested. Genetic variation was identified for 4 out of 9 disorders: mutant alleles were found in 49, 15, 3 and 2 breeds for HD, DM, EIC and NCL respectively. Additionally, mutant alleles were identified in crossbreeds for both HD and EIC. For HD, DM, EIC and NCL mutant alleles were newly discovered in 43, 13, 2 and 1 breed(s), respectively. In 9, 2 and 1 breed(s) for DM, EIC and NCL respectively, the mutant allele was detected, but the respective disorder has not been reported in those breeds. For 5 disorders (HMLR, MPS VII, MG, GM1, GRMD), the mutant allele could not be identified in our population. For the other 4 disorders (HD, DM, EIC, NCL), prevalence of associated mutant alleles seems strongly breed dependent. Surprisingly, mutant alleles were found in many breeds where the disorder has not been reported to date. PMID:24069350

  1. The Prevalence of Nine Genetic Disorders in a Dog Population from Belgium, the Netherlands and Germany

    PubMed Central

    Broeckx, Bart J. G.; Coopman, Frank; Verhoeven, Geert E. C.; Van Haeringen, Wim; van de Goor, Leanne; Bosmans, Tim; Gielen, Ingrid; Saunders, Jimmy H.; Soetaert, Sandra S. A.; Van Bree, Henri; Van Neste, Christophe; Van Nieuwerburgh, Filip; Van Ryssen, Bernadette; Verelst, Elien; Van Steendam, Katleen; Deforce, Dieter

    2013-01-01

    The objective of this study was to screen a dog population from Belgium, the Netherlands and Germany for the presence of mutant alleles associated with hip dysplasia (HD), degenerative myelopathy (DM), exercise-induced collapse (EIC), neuronal ceroid lipofuscinosis 4A (NCL), centronuclear myopathy (HMLR), mucopolysaccharidosis VII (MPS VII), myotonia congenita (MG), gangliosidosis (GM1) and muscular dystrophy (Duchenne type) (GRMD). Blood samples (K3EDTA) were collected for genotyping with Kompetitive Allele Specific PCR (n = 476). Allele and genotype frequencies were calculated in those breeds with at least 12 samples (n = 8). Hardy-Weinberg equilibrium was tested. Genetic variation was identified for 4 out of 9 disorders: mutant alleles were found in 49, 15, 3 and 2 breeds for HD, DM, EIC and NCL respectively. Additionally, mutant alleles were identified in crossbreeds for both HD and EIC. For HD, DM, EIC and NCL mutant alleles were newly discovered in 43, 13, 2 and 1 breed(s), respectively. In 9, 2 and 1 breed(s) for DM, EIC and NCL respectively, the mutant allele was detected, but the respective disorder has not been reported in those breeds. For 5 disorders (HMLR, MPS VII, MG, GM1, GRMD), the mutant allele could not be identified in our population. For the other 4 disorders (HD, DM, EIC, NCL), prevalence of associated mutant alleles seems strongly breed dependent. Surprisingly, mutant alleles were found in many breeds where the disorder has not been reported to date. PMID:24069350

  2. Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides.

    PubMed

    Mohri, Ikuko; Taniike, Masako; Okazaki, Issei; Kagitani-Shimono, Kuriko; Aritake, Kosuke; Kanekiyo, Takahisa; Yagi, Takashi; Takikita, Shoichi; Kim, Hyung-Suk; Urade, Yoshihiro; Suzuki, Kinuko

    2006-05-01

    Lipocalin-type prostaglandin (PG) D synthase (L-PGDS) is a dually functional protein, acting both as a PGD2-synthesizing enzyme and as an extracellular transporter of various lipophilic small molecules. L-PGDS is expressed in oligodendrocytes (OLs) in the central nervous system and is up-regulated in OLs of the twitcher mouse, a model of globoid cell leukodystrophy (Krabbe's disease). We investigated whether up-regulation of L-PGDS is either unique to Krabbe's disease or is a more generalized phenomenon in lysosomal storage disorders (LSDs), using LSD mouse models of Tay-Sachs disease, Sandhoff disease, GM1 gangliosidosis and Niemann-Pick type C1 disease. Quantitative RT-PCR revealed that L-PGDS mRNA was up-regulated in the brains of all these mouse models. In addition, strong L-PGDS immunoreactivity was observed in OLs, but not in either astrocytes or microglia in these models. Thus, up-regulation of L-PGDS appears to be a common response of OLs in LSDs. Moreover, surface plasmon resonance analyses revealed that L-PGDS binds GM1 and GM2 gangliosides, accumulated in neurons in the course of LSD, with high affinities (KD = 65 and 210 nm, respectively). This suggests that L-PGDS may play a role in scavenging harmful lipophilic substrates in LSD. PMID:16515539

  3. Identification of Sandhoff disease in a Thai family: clinical and biochemical characterization.

    PubMed

    Sakpichaisakul, Kullasate; Taeranawich, Pairat; Nitiapinyasakul, Achara; Sirisopikun, Todsaporn

    2010-09-01

    Sandhoff disease is a GM2 gangliosidosis that is rare in Thailand. The authors report a Thai family with two children known to have infantile form of Sandhoff disease. The index case exhibited mitral valve prolapse with mitral regurgitation as an early sign, which is a rare presentation in Sandhoff disease. Thereafter the patient had developmental regression, startle reaction, and cherry red spots. The diagnosis was confirmed by biochemical analysis. PMID:20873083

  4. Achieving patient satisfaction: resolving patient complaints.

    PubMed

    Oxler, K F

    1997-07-01

    Patients demand to be active participants on and partners with the health care team to design their care regimen. Patients bring unique perceptions and expectations and use these to evaluate service quality and satisfaction. If customer satisfaction is not achieved and a patient complaint results, staff must have the skills to respond and launch a service recovery program. Service recovery, when done with style and panache, can retain loyal customers. Achieving patient satisfaction and resolving patient complaints require commitment from top leadership and commitment from providers to dedicate the time to understand their patients' needs. PMID:9274171

  5. Hospitalized Patients and Fungal Infections

    MedlinePlus

    ... AIDS Organ transplant patients Cancer patients Hospitalized patients Stem cell transplant patients Medications that weaken your immune system ... links below: Organ Transplant Patients and Fungal Infections Stem Cell Transplant Patients and Fungal Infections Cancer Patients and ...

  6. Patient-centered Radiology.

    PubMed

    Itri, Jason N

    2015-10-01

    Patient-centered care (ie, care organized around the patient) is a model in which health care providers partner with patients and families to identify and satisfy patients' needs and preferences. In this model, providers respect patients' values and preferences, address their emotional and social needs, and involve them and their families in decision making. Radiologists have traditionally been characterized as "doctor-to-doctor" consultants who are distanced from patients and work within a culture that does not value patient centeredness. As medicine becomes more patient driven and the trajectory of health care is toward increasing patient self-reliance, radiologists must change the perception that they are merely consultants and become more active participants in patient care by embracing greater patient interaction. The traditional business model for radiology practices, which devalues interaction between patients and radiologists, must be transformed into a patient-centered model in which radiologists are reintegrated into direct patient care and imaging processes are reorganized around patients' needs and preferences. Expanding radiology's core assets to include direct patient care may be the most effective deterrent to the threat of commoditization. As the assault on the growth of Medicare spending continues, with medical imaging as a highly visible target, radiologists must adapt to the changing landscape by focusing on their most important consumer: the patient. This may yield substantial benefits in the form of improved quality and patient safety, reduced costs, higher-value care, improved patient outcomes, and greater patient and provider satisfaction. PMID:26466190

  7. Pharmacist-Patient Interaction and Patient Expectations

    ERIC Educational Resources Information Center

    DeSimone, Edward M.; And Others

    1977-01-01

    The nature of the professional interaction between the pharmacist and the patient was explored, with data collected from 3,743 questions asked of 16 pharmacists during an 8-week period. Factors that might affect the nature of the pharmacist-patient interaction are identified along with a cognitive-based profile of patient wants. (LBH)

  8. Counseling the Coronary Patient

    ERIC Educational Resources Information Center

    Semmler, Caryl; Semmler, Maynard

    1974-01-01

    The article discusses counseling sessions designed to a) help the coronary patient adjust to cardiovascular disease, b) diminish patient anxieties and fears, and c) educate the patient and family members on controlling risk factors to deter another coronary attack. (JS)

  9. Moving toward Patient Empowerment.

    ERIC Educational Resources Information Center

    Weaver, Susan K.; Wilson, Josephine F.

    1994-01-01

    Modern medical practice tends to enable patients to evade responsibility for their own care, and the traditional model robs patients of control and self-esteem. Radical health care reform must include patient empowerment and education. (SK)

  10. The Angry Dying Patient.

    PubMed

    Houston, Robert E.

    1999-02-01

    Over 25 years ago, Kubler-Ross identified anger as a predictable part of the dying process. When the dying patient becomes angry in the clinical setting, all types of communication become strained. Physicians can help the angry dying patient through this difficult time by using 10 rules of engagement. When physicians engage and empathize with these patients, they improve the patient's response to pain and they reduce patient suffering. When physicians educate patients on their normal responses to dying and enlist them in the process of family reconciliation, they can impact the end-of-life experience in a positive way. PMID:15014699

  11. The Angry Dying Patient

    PubMed Central

    Houston, Robert E.

    1999-01-01

    Over 25 years ago, Kubler-Ross identified anger as a predictable part of the dying process. When the dying patient becomes angry in the clinical setting, all types of communication become strained. Physicians can help the angry dying patient through this difficult time by using 10 rules of engagement. When physicians engage and empathize with these patients, they improve the patient's response to pain and they reduce patient suffering. When physicians educate patients on their normal responses to dying and enlist them in the process of family reconciliation, they can impact the end-of-life experience in a positive way. PMID:15014699

  12. Cancer Patients and Fungal Infections

    MedlinePlus

    ... AIDS Organ transplant patients Cancer patients Hospitalized patients Stem cell transplant patients Medications that weaken your immune system ... of getting an infection, including a fungal infection. Stem cell transplant patients or those who have a blood ( ...

  13. Periprocedural Patient Care.

    PubMed

    Kohi, Maureen P; Fidelman, Nicholas; Behr, Spencer; Taylor, Andrew G; Kolli, Kanti; Conrad, Miles; Hwang, Gloria; Weinstein, Stefanie

    2015-10-01

    Periprocedural care of patients who undergo image-guided interventions is a task of monumental importance. As physicians who perform procedures, radiologists rely on their noninterpretive skills to optimize patient care. At the center of periprocedural care is proper patient identification. It is imperative to perform the indicated procedure for the correct patient. It is also of great importance to discuss with the patient the nature of the procedure. This conversation should include the indications, risks, benefits, alternatives, and potential complications of the procedure. Once the patient agrees to the procedure and grants informed consent, it is imperative to stop and confirm that the correct procedure is being performed on the correct patient. This universal time-out policy helps decrease errors and improves patient care. To optimize our interpretative and procedural skills, it may be necessary to provide the patient with sedation or anesthesia. However, it is important to understand the continuum of sedation and be able to appropriately monitor the patient and manage the sedation in these patients. To minimize the risks of infection, periprocedural care of patients relies on aseptic or, at times, sterile techniques. Before the procedure, it is important to evaluate the patient's coagulation parameters and bleeding risks and correct the coagulopathy, if needed. During the procedure, the patient's blood pressure and at times the patient's glucose levels will also require monitoring and management. After the procedure, patients must be observed in a recovery unit and deemed safe for discharge. The fundamental components of periprocedural care necessary to enhance patient safety, satisfaction, and care are reviewed to familiarize the reader with the important noninterpretive skills necessary to optimize periprocedural care. PMID:26466184

  14. Enterotoxin production and serogroups of Campylobacter jejuni and Campylobacter coli from patients with diarrhea and from healthy laying hens.

    PubMed Central

    Lindblom, G B; Kaijser, B; Sjögren, E

    1989-01-01

    Enterotoxin production, a possible virulence factor, was determined in Campylobacter jejuni and Campylobacter coli by two different techniques, the CHO cell test and the GM1 enzyme-linked immunosorbent assay. The frequency of enterotoxigenic Campylobacter strains was 32% in strains from both humans with acute enteritis and healthy laying hens, as measured by the CHO cell test. The CHO cell test was significantly more sensitive than the GM1 enzyme-linked immunosorbent assay in the detection of enterotoxigenic strains. Enterotoxin production was compared with the presence of heat-stable and heat-labile antigens. There was no significant correlation between enterotoxin production and serogroups for C. jejuni or C. coli. The difference in enterotoxigenicity between C. jejuni (34.1%) and C. coli (21.9%) was not significant. PMID:2754001

  15. Patient Education on Pain

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  16. Repositioning the Patient:

    PubMed Central

    Mold, Alex

    2013-01-01

    Summary This article explores how and why the patient came to be repositioned as a political actor within British health care during the 1960s and 1970s. Focusing on the role played by patient organizations, it is suggested that the repositioning of the patient needs to be seen in the light of growing demands for greater patient autonomy and the application of consumerist principles to health. Examining the activities of two patient groups—the National Association for the Welfare of Children in Hospital (NAWCH) and the Patients Association (PA)—indicates that while such groups undoubtedly placed more emphasis on individual autonomy, collective concerns did not entirely fall away. The voices of patients, as well as the patient, continued to matter within British health care. PMID:23811711

  17. Multiple Myeloma: Patient Handbook

    MedlinePlus

    ... contact us TEXT SIZE IMF Multiple Myeloma Patient Handbook 2016 One of the most daunting aspects of ... with each of these steps. The IMF’s Patient Handbook is meant to furnish you with the tools ...

  18. Wooing patients with technology.

    PubMed

    Myers, Michael

    2013-04-01

    Technologies that can give healthcare organizations a marketing advantage with patients include: Registration kiosks that request payment automatically, in a more comfortable environment for both patients and registration staff. Emails that enable patients to schedule initial visits and follow-up care. Secure online messaging platforms that enable patients to obtain timely answers to questions they have for their providers both before and after receiving services. PMID:23596831

  19. Can "patient keeper" help in-patients?

    PubMed

    Al-Hinnawi, M F

    2009-06-01

    The aim of this paper is to present our "Patient Keeper" application, which is a client-server medical application. "Patient Keeper" is designed to run on a mobile phone for the client application and on a PC for the server application using J2ME and JAVA2, respectively. This application can help doctors during visits to their patients in hospitals. The client application allows doctors to store on their mobile phones the results of their diagnoses and findings such as temperature, blood pressure, medications, analysis, etc., and send this information to the server via short message service (SMS) for storage in a database. The server can also respond to any request from the client and send the result via Bluetooth, infrared, or over the air. Experimental results showed a significant improvement of the healthcare delivery and reduction for in-patient stay. PMID:19548829

  20. [Patient blood management--The preoperative patient].

    PubMed

    Mller, Markus M; Fischer, Dania; Stock, Ulrich; Geisen, Christof; Steffen, Bjrn; Nussbaumer, Judith; Meybohm, Patrick

    2014-04-01

    Preoperative anaemia is an independent risk factor for an increase in perioperative morbidity and mortality. Patient Blood Management (PBM) aims for an early detection of anaemia in elective surgery patients. Reasons for anaemia should be detected and causally treated if possible. A multidisciplinary team of specialists aims for diagnosis and causative treatment of easily treatable and frequent causes of anaemia like iron deficiency, bleeding or (autoimmune) haemolysis using patients' specific history, examination, laboratory and technical methods. Such an outpatient PBM programme is only feasible, if anaesthesiologists, surgeons, haematologists, gastroenterologists, gynecologists, laboratory and transfusion medicine specialists work together in a PBM team using a common PBM plan. Communication within this team as well as with the patients' physicians in their private offices is key for a long lasting success of such a PBM programme. PMID:24792598

  1. Patients Provide Recommendations for Improving Patient Satisfaction.

    PubMed

    Moore, Angelo D; Hamilton, Jill B; Krusel, Jessica L; Moore, LeeAntoinette G; Pierre-Louis, Bosny J

    2016-04-01

    National Committee for Quality Assurance recommends patient-centered medical homes incorporate input from patient populations; however, many health care organizations do not. This qualitative study used two open-ended questions from 148 active duty Army Soldiers and their family members to illicit recommendations for primary care providers and clinic leadership that would improve their health care experiences. Content analysis and descriptive statistics were used to analyze responses. Participant responses were related to four major themes: Access to Care, Interpersonal Interaction, Satisfaction of Care, and Quality of Care. Participants were overall satisfied with their care; however, spending less time waiting for appointments and to see the provider or specialist were the most frequently requested improvements related to Access to Care. For Interpersonal Interaction, 82% of the responses recommended that providers be more attentive listeners, courteous, patient, caring, and respectful. Decreasing wait times and improving interpersonal skills would improve health care experiences and patient satisfaction. PMID:27046182

  2. Patient-centered Care.

    PubMed

    Reynolds, April

    2009-01-01

    Patient-centered care focuses on the patient and the individual's particular health care needs. The goal of patient-centered health care is to empower patients to become active participants in their care. This requires that physicians, radiologic technologists and other health care providers develop good communication skills and address patient needs effectively. Patient-centered care also requires that the health care provider become a patient advocate and strive to provide care that not only is effective but also safe. For radiologic technologists, patient-centered care encompasses principles such as the as low as reasonably achievable (ALARA) concept and contrast media safety. Patient-centered care is associated with a higher rate of patient satisfaction, adherence to suggested lifestyle changes and prescribed treatment, better outcomes and more cost-effective care. This article is a Directed Reading. Your access to Directed Reading quizzes for continuing education credit is determined by your area of interest. For access to other quizzes, go to www.asrt.org/store. According to one theory, most patients judge the quality of their healthcare much like they rate an airplane flight. They assume that the airplane is technically viable and is being piloted by competent people. Criteria for judging a particular airline are personal and include aspects like comfort, friendly service and on-time schedules. Similarly, patients judge the standard of their healthcare on nontechnical aspects, such as a healthcare practitioner's communication and "soft skills." Most are unable to evaluate a practitioner's level of technical skill or training, so the qualities they can assess become of the utmost importance in satisfying patients and providing patient-centered care.(1). PMID:19901351

  3. Why measure patient satisfaction?

    PubMed

    Riskind, Patty; Fossey, Leslie; Brill, Kari

    2011-01-01

    A practice that consistently and continuously measures patient perceptions will be more efficient and effective in its daily operations. With pay-for-performance requirements on the horizon and consumer rating sites already publicizing impressions from physician encounters, a practice needs to know how it is performing through the eyes of the patients. Azalea Orthopedics has used patient feedback to coach its physicians on better patient communication. The Orthopaedic Institute has used patient satisfaction results to reduce wait times and measure the return on investment from its marketing efforts. Patient survey results that are put to work can enhance the efficiency and effectiveness of practice operations as well as position the practice for increased profitability. PMID:21506460

  4. Patient-doctor communication.

    PubMed

    Teutsch, Carol

    2003-09-01

    Communication is an important component of patient care. Traditionally, communication in medical school curricula was incorporated informally as part of rounds and faculty feedback, but without a specific or intense focus on skills of communicating per se. The reliability and consistency of this teaching method left gaps, which are currently getting increased attention from medical schools and accreditation organizations. There is also increased interest in researching patient-doctor communication and recognizing the need to teach and measure this specific clinical skill. In 1999, the Accreditation of Council for Graduate Medical Education implemented a requirement for accreditation for residency programs that focuses on "interpersonal and communications skills that result in effective information exchange and teaming with patients, their families, and other health professionals." The National Board of Medical Examiners, Federation of State Medical Boards. and the Educational Commission for Foreign Medical Graduates have proposed an examination between the. third and fourth year of medical school that "requires students to demonstrate they can gather information from patients, perform a physical examination, and communicate their findings to patients and colleagues" using standardized patients. One's efficiency and effectiveness in communication can be improved through training, but it is unlikely that any future advances will negate the need and value of compassionate and empathetic two-way communication between clinician and patient. The published literature also expresses belief in the essential role of communication. "It has long been recognized that difficulties in the effective delivery of health care can arise from problems in communication between patient and provider rather than from any failing in the technical aspects of medical care. Improvements in provider-patient communication can have beneficial effects on health outcomes". A systematic review of randomized clinical trials and analytic studies of physician-patient communication confirmed a positive influence of quality communication on health outcomes. Continuing research in this arena is important. For a successful and humanistic encounter at an office visit, one needs to be sure that the patient's key concerns have been directly and specifically solicited and addressed. To be effective, the clinician must gain an understanding of the patient's perspective on his or her illness. Patient concerns can be wide ranging, including fear of death, mutilation, disability; ominous attribution to pain symptoms; distrust of the medical profession; concern about loss of wholeness, role, status, or independence; denial of reality of medical problems; grief; fear of leaving home; and other uniquely personal issues. Patient values, cultures, and preferences need to be explored. Gender is another element that needs to be taken into consideration. Ensuring key issues are verbalized openly is fundamental to effective patient-doctor communication. The clinician should be careful not to be judgmental or scolding because this may rapidly close down communication. Sometimes the patient gains therapeutic benefit just from venting concerns in a safe environment with a caring clinician. Appropriate reassurance or pragmatic suggestions to help with problem solving and setting up a structured plan of action may be an important part of the patient care that is required. Counseling around unhealthy or risky behaviors is an important communication skill that should be part of health care visits. Understanding the psychology of behavioral change and establishing a systematic framework for such interventions, which includes the five As of patient counseling (assess, advise, agree, assist, and arrange) are steps toward ensuring effective patient-doctor communication. Historically in medicine, there was a paternalistic approach to deciding what should be done for a patient: the physician knew best and the patient accepted the recommendation without question. This era is ending, being replaced with consumerism and the movement toward shared decision-making. Patients are advising each other to "educate yourself and ask questions". Patient satisfaction with their care, rests heavily on how successfully this transition is accomplished. Ready access to quality information and thoughtful patient-doctor discussions is at the fulcrum of this revolution. PMID:14621334

  5. Patient Education: An Annotated Bibliography.

    ERIC Educational Resources Information Center

    Simmons, Jeannette

    Topics included in this annotated bibliography on patient education are (1) background on development of patient education programs, (2) patient education interventions, (3) references for health professionals, and (4) research and evaluation in patient education. (TA)

  6. Histoplasmosis in immunosuppressed patients.

    PubMed

    Kauffman, C A; Israel, K S; Smith, J W; White, A C; Schwarz, J; Brooks, G F

    1978-06-01

    Infection with Histoplasma capsulatum in 58 patients whose immune responses were suppressed (Immunosuppressed patients) (16 from the present series and 42 described previously) was analyzed. The most common underlying diseases were Hodgkin's disease (29 per cent), chronic lymphocytic leukemia (19 per cent) and acute lymphocytic leukemia (17 per cent). Sixty-three per cent of the patients had received cytotoxic drugs, and 57 per cent had taken corticosteroids. Widely disseminated infection occurred in 88 per cent of the patients, with predominant involvement of lungs and organs of the reticuloendothelial system. Localized pulmonary infection was present in the remaining patients. The most useful diagnostic method was bone marrow biopsy with microscopic examination for the intracellular yeast form of H. capsulatum. Biopsy of oral lesions, lung, liver and lymph node also proved diagnostically helpful. Growth of H. capsulatum in culture was frequently too slow to be beneficial in diagnosing histoplasmosis in ill patients. Serologic methods were of little diagnostic help in this population of immunosuppressed patients. The response to amphotericin B therapy was excellent (6.7 per cent mortality rate) in those patients in whom the diagnosis was established early and in whom a full course of antifungal therapy could be given. In contrast, the mortality rate in patients who received no antifungal therapy or less than 1 g of amphotericin B was 100 per cent. PMID:350045

  7. The critically ill immunosuppressed patient

    SciTech Connect

    Parrillo, J.E.; Masur, H. )

    1987-01-01

    This book discusses the papers on the diagnosis and management of immunosuppressed patient. Some of the topics are: life-threatening organ failure in immunosuppressed patients; diagnosis and therapy of respiratory disease in the immunosuppressed patient; CNS complication of immunosuppression; infections; antineoplastic therapy of immunosuppressed patient; radiation therapy-issues in critically ill patient; AIDS; and management of bone marrow transplant patients.

  8. The dying thoracic patient.

    PubMed

    Krishna, G; Raffin, T A

    1998-08-01

    Health care providers should understand that the practice of good medicine includes not only diagnosing and curing diseases, but also effectively communicating with patients and families and helping terminally ill patients die a peaceful and dignified death. Patients in America come from varied backgrounds, and it is important for physicians to consider cultural and religious issues. Physicians should combine their clinical judgment with objective outcome data to provide optimal care for patients. Informed consent should be obtained from patients after offering a detailed plan of care that would include appropriate interventions and the consequences of no intervention. The physician should then assist the patient in making a decision that would provide the best possible future for that individual. The four fundamental principles of biomedical ethics, namely beneficence, nonmaleficence, autonomy, and justice, should be considered when analyzing an ethical problem. Voluntary active euthanasia, which means performing a deliberate act (e.g., administering a lethal injection) to end a patient's life, should not be performed by a physician. Withholding and withdrawing basic and advanced life support constitutes passive euthanasia. Good communication with patients early in the clinical course whenever possible results in an ethically correct decision. A nonconfrontational, sympathetic, and compassionate approach to family members and legal surrogates facing the immediate death of their loved ones leads to the best possible outcome. It is the duty of the physician to assure the patient and the family that he or she will not abandon the patient. Effective communication is the key to solving almost all ethical dilemmas when caring for the dying thoracic patient. PMID:9742345

  9. The Patient as Teacher.

    ERIC Educational Resources Information Center

    Weisser, Roland J.; Medio, Franklin J.

    1985-01-01

    A program of seminars designed to explore student perceptions of the emotional and psychological aspects of physician-patient interactions, part of a clinical diagnosis course, used graduate medical students to act as patients in videotaped interviews, interview evaluations, and lectures and demonstrations on interpersonal communication skills.…

  10. Patient Education Thesaurus.

    ERIC Educational Resources Information Center

    Cooper, Lynn

    This thesaurus was compiled to make the materials in the Patient Education Room of the Donald J. Vincent Medical Library at Riverside Methodist Hospital, Columbus, Ohio, more accessible to patients. Subjects are grouped in fairly broad categories (e.g., Aging & Problems of Aging; Alcohol & Alcohol Abuse; Careers in the Medical Field; Childhood and

  11. Communicating with patients.

    PubMed

    Kaplowitz, G J

    1999-01-01

    Dental care providers need to be able to communicate effectively with their patients in order to build rapport and trust. Highly developed communication skills also enable the dental care provider to extract more accurate diagnostic information and to more effectively present treatment options to the patient. Neurolinguistic programming techniques can be employed to accomplish these as well as other objectives. PMID:10687469

  12. Management of elderly patients

    PubMed Central

    Corre, Romain; Lena, Hervé; Le Caer, Hervé

    2013-01-01

    Summary Elderly patients are often excluded from clinical trials, yet more than two-thirds of patients diagnosed with lung cancer are over 65 years old. It is therefore important to develop specific tools and trials for this specific patient population. Methods This chapter first examines the management specificities of elderly patients. Randomized trials specifically involving elderly patients are then described, and likely future developments are considered. Results Older people have several specificities. In addition to traditional criteria such as age and performance status, other important factors include the number of comorbidities and age-related changes such as cognitive deficits and depression. Specific indices taking these factors into account have been published and validated. Single-agent therapy has been widely used to treat metastatic lung cancer in the elderly, following publication of negative results from randomized phase III trials of combination chemotherapy. Recently, however, a trial of doublet therapy gave positive results, in a subgroup of independent older patients. The benefit of patient selection based on a combination of these indices has been demonstrated in open-label and randomized trials. These results must now be confirmed in phase III trials including the use of tyrosine kinase inhibitors combined with chemotherapy. Conclusions Indices based on a combination of age-related factors, together with judicious use of biological markers, will further improve the prognosis of elderly lung cancer patients. PMID:25806233

  13. Patient Education Thesaurus.

    ERIC Educational Resources Information Center

    Cooper, Lynn

    This thesaurus was compiled to make the materials in the Patient Education Room of the Donald J. Vincent Medical Library at Riverside Methodist Hospital, Columbus, Ohio, more accessible to patients. Subjects are grouped in fairly broad categories (e.g., Aging & Problems of Aging; Alcohol & Alcohol Abuse; Careers in the Medical Field; Childhood and…

  14. Patient Education in Thyrotoxicosis.

    ERIC Educational Resources Information Center

    Lincoln, N. B.; Sturrock, N. D. C.; Sowter, H.; Abbott-Harland, S.; Nichols, E.; Jeffcoate, W. J.

    2000-01-01

    Study aims to assess the need for a thyrotoxicosis patient education programs and evaluates a group education session. Patients with thyrotoxicosis were surveyed to assess their needs. Determined that people with thyrotoxicosis had limited knowledge about their condition. The offer of a group education program has little effect on that knowledge…

  15. Approach to bleeding patient

    PubMed Central

    Gopinath, Ramachandran; Sreekanth, Y.; Yadav, Monu

    2014-01-01

    Managing a bleeding patient is very challenging for the perioperative physician. Bleeding in a patient would be due to inherited or acquired disorders of haemostasis. Identifying the patients at risk of bleeding and utilising prophylactic treatment protocols has good outcomes. Along with clinical signs, trends in monitoring coagulation parameters and analysing blood picture are necessary. Management of patients in the postoperative period and in intensive care unit should be focused on normalization of coagulation profile as early as possible with available blood and its products. Available recombinant factors should be given priority as per the approved indications. Exploring the surgical site should be considered for persistent bleeding because haemodynamic compromise, excessive transfusion of fluids, blood and its products and more inotropic support may have a negative impact on the patient outcome. PMID:25535422

  16. Doctor-patient relationship

    PubMed Central

    Chamsi-Pasha, Hassan; Albar, Mohammed A.

    2016-01-01

    The doctor-patient relationship is an intricate concept in which patients voluntarily approach a doctor and become part of a contract by which they tend to abide by doctor’s instructions. Over recent decades, this relationship has changed dramatically due to privatization and commercialization of the health sector. A review of the relevant literature in the database of MEDLINE published in English between 1966 and August 2015 was performed with the following keywords: doctor-patient relationship, physician-patient relationship, ethics, and Islam. The Muslim doctor should be familiar with the Islamic teachings on the daily issues faced in his/her practice and the relationship with his/her patients. PMID:26837392

  17. [The expert patient: medical consequences].

    PubMed

    Nagel, G

    2006-12-01

    The expression expert patient appeared about ten years ago. It defines the role of patients who are actively involved in their disease management. Most clinical oncologists are challenged by expert patients. Patient's expertise and complementary medicine are closely linked. Physicians respecting expert patients have to find a positive attitude towards patient self aide concepts. The medical skill to manage expert patients is increasingly important and new tools are being developed for support. PMID:17213970

  18. Nutrition in dialysis patients.

    PubMed

    Sen, D; Prakash, J

    2000-07-01

    Malnutrition is a common clinical problem in dialysis patients, which is multifactorial in origin. It is most often found in a patient of chronic renal failure (CRF) during the period when the glomerular filtration rate (GFR) falls below 10 ml/min, but dialysis is yet to be started. The loss of proteins, aminoacids and other essential nutrients during the procedure of dialysis may further aggravate the malnutrition. Poor nutrition in dialysis patients is associated with increased morbidity and mortality in the form of delayed wound healing, malaise, fatigue, increased susceptibility to infection and poor rehabilitation. In view of the above consequences, all patients on dialysis must undergo nutritional assessment. It is very vital to maintain good nutritional status in-patients on dialysis by adequate protein and calories intake, appropriate supplementation of iron, calcium, minerals and water-soluble vitamins and, of course, the supplementation should be individualised. Nutritional needs are enhanced in presence of stresses like infection or surgery to limit excessive tissue catabolism and therefore, these are the situations, which demand intensive nutrition therapy. Total parenteral nutrition (TPN) may be required for patients on dialysis in intensive care unit, using a central venous catheter. However, enteral route is always preferred to parenteral ones, whenever possible. Even after adequate dialysis has been given, dietary counselling is often required for both hemodialysis and peritoneal dialysis patients to ensure that they ingest the recommended amount of protein, calories and essential micronutrients. PMID:11273510

  19. The medically compromised patient.

    PubMed

    Parnell, A G

    1986-06-01

    Diabetes mellitus is found in up to 5 per cent of the population. There is an excess of blood sugar due to a deficiency or diminished effectiveness of insulin. It is a complex disease which, if not controlled, has many major complications including an increased incidence of heart attacks, strokes and vascular changes in many other organs. The management of young onset diabetic patients is directed towards: controlling the carbohydrate intake, testing the blood sugar by the patient and regular insulin injections. Great care must be taken in treating diabetics in the dental surgery. Except for children, any diabetic can be treated for simple dental procedures by ensuring freedom from pain, by eliminating stress and by ensuring that the patient does not miss a meal. Children, unstable diabetic patients and those with infections or requiring multiple extractions should be treated in hospital under the care of an endocrinologist. In hypertension it is only after a number of years that complications begin to appear. The main ones are those of stroke, retinal haemorrhages, renal failure and heart disease. Dentists should be encouraged to take the blood pressure of all adults who present for treatment. Patients with increased blood pressure yet controlled by drugs may be treated as normal patients. Those that are not well controlled should be referred to their physician. Dental appointments must be free of pain and stress should be avoided. A screening method is presented which assists in the evaluation of medically compromised patients. PMID:2941377

  20. Rethinking patient education.

    PubMed

    Luker, K; Caress, A L

    1989-09-01

    The aim of this paper is to critically examine and challenge some of the assumptions which underpin the research and non-research based literature on patient education. Doubts are expressed concerning the transferability of theories of adult learning to patient education; and concern is expressed over the imbalance in the literature where emphasis is placed on the psychological benefits of teaching, rather than physical outcomes. In the light of the available evidence which suggests that nurses are not 'good patient teachers' the case is made to support the suggestion that patient education should become the responsibility of specialist nurses. In addition, computer-assisted learning (CAL) is proffered as the solution to a number of the problems facing patient educators. CAL is seen as a means of empowering the patient, rather than the nurse to take control, and this is viewed as a positive move in the direction of self-care. The paper concludes by suggesting that CAL might be used with good effect by patients with particular learning difficulties; for example the blind or partially sighted, and people who are illiterate or have a low reading ability. PMID:2674244

  1. Do patients have duties?

    PubMed Central

    Evans, H M

    2007-01-01

    The notion of patients' duties has received periodic scholarly attention but remains overwhelmed by attention to the duties of healthcare professionals. In a previous paper the author argued that patients in publicly funded healthcare systems have a duty to participate in clinical research, arising from their debt to previous patients. Here the author proposes a greatly extended range of patients' duties grounding their moral force distinctively in the interests of contemporary and future patients, since medical treatment offered to one patient is always liable to be an opportunity cost (however justifiable) in terms of medical treatment needed by other patients. This generates both negative and positive duties. Ten duties—enjoining obligations ranging from participation in healthcare schemes to promoting one's own earliest recovery from illness—are proposed. The characteristics of these duties, including their basis, moral force, extent and enforceability, are considered. They are tested against a range of objections—principled, societal, epistemological and practical—and found to survive. Finally, the paper suggests that these duties could be thought to reinforce a regrettably adversarial characteristic, shared with rights‐based approaches, and that a preferable alternative might be sought through the (here unexplored) notion of a “virtuous patient” contributing to a problem‐solving partnership with the clinician. However, in defining and giving content to that partnership, there is a clear role for most, if not all, of the proposed duties; their value thus extends beyond the adversarial context in which they might first be thought to arise. PMID:18055897

  2. Respecting patient confidentiality.

    PubMed

    Price, Bob

    2015-02-01

    Nurses face a particular challenge in respecting the confidentiality of patients in a world where information is quickly shared and where information about illness can be sensitive. We have a duty of care towards patients. That duty includes maintaining privacy (protecting them from undue intrusion), and confidentiality (by the discreet management of information about themselves that they share with us). Legislation on confidentiality comes from different sources and should be interpreted in the clinical setting. This article summarises the principal requirements set out in the legislation and directs readers to questions and tools designed to help them explore the extent to which patient confidentiality is respected where they work. PMID:25627535

  3. Psychotherapist, Psychiatrist, and Patient

    PubMed Central

    PILOWSKY, DANIEL; BELLINSON, JILL

    1996-01-01

    An increasing number of psychiatrists consult to nonmedical psychotherapists and treat their patients with medication. This dual treatment arrangement is known as "split treatment." Split treatment arrangements generate a triangular relationship among the three parties—patient, psychotherapist, and psychiatrist. Both the transferential aspects of the triangular relationship among the two therapists and a patient and the overt interpersonal conflicts that may arise—such as ideological and interdisciplinary conflicts—are discussed. Enhanced awareness of both sets of factors may facilitate the cooperation between psychiatrists and psychotherapists. PMID:22700262

  4. Why make people patients?

    PubMed Central

    Marinker, Marshall

    1975-01-01

    People confront their doctors with three modes of unhealth - disease, illness and sickness. Each is discussed, and the question is asked and answered as to why in this situation people wish to become patients. PMID:1177270

  5. Working with Patients: Diagnosis

    MedlinePlus

    ... method of detecting asbestos-related disorders. 3. Pulmonary function tests A patient performs a pulmonary function test. ... times greater for men than for women. Lung Function Test Findings Lung function tests (spirometry) were offered ...

  6. Doctors and patients.

    PubMed

    Gillon, R

    1986-02-15

    Gillon outlines some prima facie moral duties of physicians to patients that have emerged from his previous articles in a series on philosophical medical ethics. These duties follow from four general ethical principles--respect for autonomy, beneficence, non-maleficence, and justice--plus the self-imposed supererogatory duty of medical beneficence. From these principles the author derives such duties as providing adequate information and advice on treatment options, encouraging patient participation leading to informed decisions, maintaining competence and exposing incompetence, admitting errors, disclosing personal medico-moral standards, and acknowledging that other interests may occasionally supersede those of the individual patient. Gillon concludes that, where self interest conflicts with medical beneficence, the claim of medicine as a profession requires that the patient's interests take priority. PMID:3081128

  7. [Rehabilitation for cancer patients].

    PubMed

    Tanuma, Akira

    2013-09-01

    In Japan, the number of patients with cancer is increasing drastically with the increase in number of elderly people. Therefore, recently, the necessity of rehabilitation for cancer patients has been realized. Cancer rehabilitation can be classified as preventive, restorative, supportive, or palliative and is administered according to the degree of cancer progression. Rehabilitation is of great significance even for patients with progressive cancer as it helps maintain their quality of life. Various forms of impairment, disability, and handicap are associated with cancer rehabilitation. Examples of impairments that cancer patients experience are hemiplegia and higher brain dysfunction in brain tumor cases, paraplegia and quadriplegia in spinal or spinal cord tumor cases, neuropathy and radiculopathy in cases of tumor invasion, complications after surgery, peripheral neuropathy after chemotherapy, and dysphagia after radiotherapy. It is important to evaluate these impairments and the risks associated with rehabilitation. PMID:24047769

  8. National Patient Safety Foundation

    MedlinePlus

    ... Awareness Alert BMJ Quality & Safety Resource Guides PLS Webcast Archives Stand Up Templates and Logos Patient Safety ... Oversight Committee CBPPS Board of Directors Education & Resources Webcasts Upcoming Webcasts Professional Learning Series Webcasts CPPS Review ...

  9. [Patient safety: Glossary].

    PubMed

    Sabio Paz, Verónica; Panattieri, Néstor D; Cristina Godio, Farmacéutica; Ratto, María E; Arpí, Lucrecia; Dackiewicz, Nora

    2015-10-01

    Patient safety and quality of care has become a challenge for health systems. Health care is an increasingly complex and risky activity, as it represents a combination of human, technological and organizational processes. It is necessary, therefore, to take effective actions to reduce the adverse events and mitigate its impact. This glossary is a local adaptation of key terms and concepts from the international bibliographic sources. The aim is providing a common language for assessing patient safety processes and compare them. PMID:26294153

  10. Patient Attitudes to Tonsillectomy

    PubMed Central

    Ubayasiri, Kishan; Kothari, Ravi; McClelland, Lisha; De, Mriganka

    2012-01-01

    Introduction. Recent changes to primary care trusts' Procedures of Limited Clinical Value (PLCV) policy mean that otolaryngologists must now follow policy rather than exercising clinical judgment when listing patients for tonsillectomy. Objectives. To gauge perception within the general public of when tonsillectomy is acceptable and to compare this to the current policy. Method. All patients or their parents attending the adult and paediatric outpatient ENT departments were asked to anonymously complete questionnaires. Results. One hundred and twenty-five completed questionnaires were collected. Thirty-one percent of respondents thought tonsillectomy should be offered solely on patient request, 19% after one to three bouts, and 35% after four to six bouts of tonsillitis. Only 9% thought the current guidelines were reasonable. Patients who had suffered recurrent tonsillitis or had undergone previous tonsillectomy generally thought tonsillectomy advisable after more bouts of tonsillitis than those who had not. Fourteen patients fulfilled the SIGN guidelines for tonsillectomy for recurrent tonsillitis. Of these, 13 (93%) felt that suffering 4–6 bouts of tonsillitis was reasonable before tonsillectomy. Conclusion. All patients we surveyed who meet the current PLCV and SIGN guidelines regarding the appropriateness of tonsillectomy for recurrent tonsillitis perceive that they are excessive, believing that 4–6 bouts of recurrent tonsillitis are adequate to justify tonsillectomy. PMID:23320167

  11. [Urosepsis in Geriatric Patients].

    PubMed

    Heppner, H J; Yapan, F; Wiedemann, A

    2016-02-01

    Due to the demographic shift, increasing numbers of geriatric patients are admitted to acute care hospitals of all levels of care. This means that special challenges must be met in the medical care and management of these patients.Immunosenescence and multimorbidity make elderly patients vulnerable to infectious diseases. Urinary tract infections range from "simple" cystitis to pyelonephritis and urosepsis and, at 25%, are the second most common form of infection in geriatric patients. It is often difficult to make a diagnosis because typical symptoms do not always occur. Urosepsis, a hyperactive and uncontrolled immune response of the organism due to exogenous damage, is based on bacterial infection of the urogenital tract. Urinary retention, immunosuppressive medication, malignancy, diabetes mellitus and renal or prostatic processes promote the risk for urosepsis. Complicated urosepsis additionally comprises a structural or functional abnormality, including ureteral obstruction. Risk factors for urosepsis are urinary incontinence, an indwelling urinary catheter, hydronephrosis or ureteral calculi. Patients suffering from diabetes mellitus are also at a higher risk for urosepsis. When diagnosing elderly patients, one has to consider that the classic symptoms can be masked by multimorbidity, or septic encephalopathy and acute confusion (delirium) may be the only symptoms. Body temperature is lower in elderly patients and does not necessarily rise to 38°C or more in the acute phase. In patients older than 75 years who are suspicious for sepsis, temperatures as low as 37.4°C should be rated as fever. Treatment of urosepsis basically includes clearing the focus, antimicrobial treatment, stabilisation of circulation and replacement of failed organ functions. Initial empiric antibiotic treatment, depending on local resistance, should be done with acylaminopenicilline and beta-lactamase inhibitors (e. g. piperacillin/combactam or tazobactam or group 3 cephalosporins (e. g. cefotaxim). In case of intolerance, fluoroquinolone with high urinary excretion or carbapenem can be used. Also multidrug resistant germs are of importance for urosepsis and require appropriate initial antibiotic treatment.The multimorbidity of geriatric patients puts them at risk for a severe course of infectious diseases. Early identification of high-risk patients and geriatric expert monitoring in intensive care units may assist intensive care physicians. Treatment success in intensive care can be maintained by early geriatric acute rehabilitation. This requires all those involved to enter into an interdisciplinary and interprofessional dialogue. PMID:26916045

  12. [Patient-controlled analgesia].

    PubMed

    Scherpereel, P

    1991-01-01

    Patient controlled analgesia (PCA) is a drug delivery system aimed to control acute pain using negative feedback technology in a closed loop system in which the patient plays an active role. It overcomes the inadequacies of traditional analgesic protocols due to marked differences in pharmacokinetic and dynamy of analgesis between patients. Moreover, doctors and nurses frequently underprescribe opioids in patients with severe pain for fear of dangerous side-effects. A safe and effective delivery of these drugs on patient demand can be achieved using various delivery systems, modes and dosing parameters. Most devices provide both demand dosing, where a constant predetermined dose is self administered, and constant rate infusion plus demand dosing, where the minimum administration rate is determined by the doctor, but can be supplemented by patient demand. Morphine sulphate remains the drug most commonly used in PCA therapy, but meperidine hydrochloride, alfentanil, nalbuphine and buprenorphine are also sometimes administered. The doctor determines the incremental dose per demand, the lockout interval, and the maximum dose per time unit, possibly also the loading dose and the minimum dose rate when a continuous flow is used. PCA provides improved analgesia, which is immediate and independent of nurse availability. This technique decreases opioid requirements, and the required total amounts are lowered. PCA gives patients both behavioural and decisional control. They can titrate the analgesic dose in such a way as to balance pain relief with the degree of side-effects, the patient is willing to tolerate. Patients often choose less than the available total dose of analgesic. The risks consists in the usual opioid side-effects, mainly respiratory depression. These may be due to mechanical problems, machine failure, or user incidents (misprogramming, or miscalculation of doses). Standards help to ensure consistent care and avoid errors that can occur even with handwritten orders. The principles of demand analgesia are now being investigated using other agents, such as local anaesthetics, and other routes of administration, mainly epidural injection. In most patients, even in children, PCA can replace intramuscular injections, which are the standard route for opioid administration. Today PCA and spinal opioids are the two main methods of analgesia for postoperative pain management. PMID:1854055

  13. Choosing effective patient education materials

    MedlinePlus

    Your patient's preferences can guide your choice of education materials and methods. Find out how your patient ... aware that you may need to adjust your education plan based on the patient's health status and ...

  14. Plastic Surgery for Ethnic Patients

    MedlinePlus

    ... Home > News > Plastic Surgery for Ethnic Patients Briefing Paper: Plastic Surgery for Ethnic Patients More than 3. ... 2067-2071. Share Related Links Plastic Surgery Briefing Papers Menu Cosmetic Reconstructive Patient Safety Before & After Find ...

  15. Transporting Forensic Psychiatric Patients.

    PubMed

    Dike, Charles C; Nicholson, Elizabeth; Young, John L

    2015-12-01

    Patients in a forensic psychiatric facility often require escorted transport to medical facilities for investigations or treatments of physical health ailments. Transporting these patients presents significant safety and custody challenges because of the nature of patients housed in forensic psychiatric facilities. A significant proportion of these patients may be transfers from the Department of Corrections (DOC) under legal mandates for psychiatric evaluation and treatment better provided in a hospital setting, and most of them will return to the DOC. Although departments of correction have protocols for escorting these potentially dangerous individuals, it is unclear whether receiving psychiatric hospitals have established procedures for maintaining the safety of others and custody of these individuals during transportation outside the hospital facility. The literature is sparse on precautions to be observed when transporting dangerous forensic psychiatric patients, including those with high escape risk. In this article, we describe one forensic inpatient facility's procedure for determining the appropriate level needed to transport these individuals outside of the forensic facility. We also describe the risk assessment procedure for determining level of transport. These are quality improvement measures resulting from a critical review of an incident of escape from the forensic facility several years ago. PMID:26668224

  16. COPD: the patient perspective.

    PubMed

    Jones, Paul W; Watz, Henrik; Wouters, Emiel Fm; Cazzola, Mario

    2016-01-01

    Chronic obstructive pulmonary disease (COPD) is a highly prevalent disease characterized by nonreversible airway obstruction. Well-characterized symptoms such as exertional dyspnea and fatigue have a negative impact on patients' quality of life (QoL) and restrict physical activity in daily life. The impact of COPD symptoms on QoL is often underestimated; for example, 36% of patients who describe their symptoms as being mild-to-moderate also admit to being too breathless to leave the house. Additionally, early morning and nighttime symptoms are a particular problem. Methods are available to allow clinicians to accurately assess COPD symptoms, including patient questionnaires. Integrated approaches to COPD management, particularly pulmonary rehabilitation, are effective strategies for addressing symptoms, improving exercise capacity and, potentially, also increasing physical activity. Inhaled bronchodilators continue to be the mainstay of drug therapy in COPD, where options can be tailored to meet patients' needs with careful selection of the inhaled medication and the device used for its delivery. Overall, an integrated approach to disease management should be considered for improving QoL and subsequent patient outcomes in COPD. PMID:26937186

  17. [Toxoplasmosis in immunocompromised patients].

    PubMed

    Machala, L; Kodym, P; Malý, M; Geleneky, M; Beran, O; Jilich, D

    2015-06-01

    In humans, toxoplasmosis mostly occurs as a latent infection, but in immunocompromised individuals, the agent may reactivate and cause severe to life-threatening disease. HIV positive individuals and transplant recipients, in particular hematopoietic stem cell transplant and heart transplant recipients, are at highest risk. The disease most often affects the central nervous system but can involve any organ. Because of the alteration of the immune response in these patients, the serodiagnosis is not reliable and direct detection of the causative agent is needed--namely by microscopy and DNA PCR. If inadequately treated or left untreated, toxoplasmosis generally has a fatal prognosis in immunocompromised patients and therefore, the treatment must be started as early and energetically as possible. The gold standard both in the treatment of reactivation and secondary prophylaxis is the pyrimethamine-sulfadiazine combination while co-trimoxazole can be used in the primary prophylaxis for high-risk patients. PMID:26099608

  18. [Respecting patient intimacy].

    PubMed

    2014-04-01

    Transparency as a general rule for all our professional acts casts doubts about the statement of the Hippocratic Oath that says "Whatever I see or hear in the lives of my patients, I will keep secret, as considering all such things to be private". Medical secrecy protects the intimacy of patients, who reveal to their physicians their most hidden secrets aiming to recover their health. Therefore, physicians should receive those secrets with reverence and care, as servers and not as their owners. The values associated with the respect for personal intimacy are the anthropological basis of medical confidentiality. A medical act is performed by definition between two equally honorable individuals. Therefore, the professional honors the trust of his patient, maintaining strict confidence of what is revealed. Therefore, medical secrecy must be strengthened rather than weakened, pursuing common wealth and dignity. PMID:25117042

  19. Mental patients in prisons

    PubMed Central

    ARBOLEDA-FLÓREZ, JULIO

    2009-01-01

    Mental conditions usually affect cognitive, emotional and volitional aspects and functions of the personality, which are also functions of interest in law, as they are essential at the time of adjudicating guilt, labeling the accused a criminal, and proffering a sentence. A relationship between mental illness and criminality has, thus, been described and given as one of the reasons for the large number of mental patients in prisons. Whether this relationship is one of causality or one that flows through many other variables is a matter of debate, but there is no debating that prisons have become a de facto part, and an important one, of mental health systems in many countries. This paper deals with the issue of the relationship and provides estimates of prevalence of mental patients in prisons culled from many studies in different countries. It also provides some direction for the management of mental patients as they crowd correctional systems. PMID:19812758

  20. Patient access innovations: integrating patients within the system of care.

    PubMed

    Marino, Daniel J; Faber, William; Duncan, Meredith

    2015-12-01

    Clinically integrated networks seeking to ensure in-network access and strengthen patient engagement should adopt five strategic areas of focus: Extend access beyond traditional models. Manage out-migration. Make it easy for patients to stay in the network. Build patient engagement into clinical care models. Explore innovative methods to engage patients. PMID:26793943

  1. Patient Portals: An Underused Resource for Improving Patient Engagement.

    PubMed

    Tulu, Bengisu; Trudel, John; Strong, Diane M; Johnson, Sharon A; Sundaresan, Devi; Garber, Lawrence

    2016-01-01

    The potential of patient portals to improve patient engagement and health outcomes has been discussed for more than a decade. The slow growth in patient portal adoption rates among patients and providers in the United States, despite external incentives, indicates that this is a complex issue. We examined evidence of patient portal use and effects with a focus on the pulmonary domain. We found a paucity of studies of patient portal use in pulmonary practice, and highlight gaps for future research. We also report on the experience of a pulmonary department using a patient portal to highlight the potential of these systems. PMID:26066707

  2. Patient-centered communication.

    PubMed

    Brown, S J

    1999-01-01

    The term patient-centered communication (PCC) has been used to describe a group of communication strategies and behaviors that promote mutuality, shared understandings, and shared decision making in health care encounters. There is evidence to suggest that advanced practice nurse and patients use these strategies to co-produce highly individualized clinical discourse. Although the communication behaviors associated with PCC have been studied separately, their impact as an integrated communications strategy has not been studied. Suggestions for developing PCC as a mid-range theory of health care communication encompassing other more specific communication concepts are offered. PMID:10418654

  3. [Anesthesia for ambulatory patients].

    PubMed

    Landauer, B

    1975-11-13

    The specific problems of outpatient anesthesia are discussed with respect to the patient's condition, the anesthesist's qualification and pharmacological properties of anesthetics used. Methohexitone seems to be the best choice for induction. Problems may arise from the use of Propanidid, Ketamin and Diazepam. Nitrousoxide and Enflurane are a suitable completion. Endotracheal intubation, if needed, is facilitated by Suxamethonium, which is rapidly eliminated. Practical aspects of timing, premedication, induction, maintenance and ending of anesthesia are pointed out. After 1-2 hours the patient can be allowed to leave the hospital accompanied by a responsible person. Driving a car is not recommended before 24 hours have elapsed since anesthesia. PMID:1220997

  4. Awareness of osteoporotic patients.

    PubMed

    Kutsal, Yeşim Gökçe; Atalay, Ayçe; Arslan, Sule; Başaran, Aynur; Cantürk, Ferhan; Cindaş, Abdullah; Eryavuz, Merih; Irdesel, Jale; Karadavut, Kiymet-Ikbal; Kirazli, Yeşim; Sindel, Dilşad; Senel, Kazim; Güler-Uysal, Füsun; Yildirim, Kadir

    2005-02-01

    Osteoporosis has recently been recognized as a major public health problem by some governments and health care providers. Despite significant progress in knowledge about osteoporosis, public awareness is required for effective management if precautions are to be taken. The aim of this study was to evaluate the educational status of osteoporotic individuals, and their awareness about the disease and sources of information. We also aimed to compare the variables between the geographical regions of the country, since significant differences exist in socioeconomic status and lifestyle within the same cultural context. This multicenter study was carried out in eight cities located in six different geographical regions between September 2001 and January 2002. To our knowledge, this is the first multicenter study in Turkey evaluating the relationship between awareness of osteoporotic patients and other variables such as educational level and economic factors. Ten centers took part in this study and consecutive patients with either femoral or lumbar T-scores below -2.5 SD were enrolled into the study group. Bone mineral density was measured using dual energy X-ray absorptiometry (DXA). Patients were interviewed using a questionnaire on past medical history, education, awareness about their disease and risk factors for osteoporosis. A total of 540 women (93.8%) and 36 men (6.3%) were included in the study. Fifty-four percent of patients declared that they were aware of osteoporosis. With regard to sources of information, 56.8% of patients reported physicians as the main source of information. Awareness of the patients was negatively correlated with age (P=0.025, r=-0,94) but positively correlated with education (P=0.0001, r=0.327), level of physical activity (P=0.001, r=143), calcium intake (P=0.005, r=119) and modern clothing style (P=0.0001, r=309). Educational status of the patients was negatively correlated with BMI (P=0.0001, r=-283) and positively correlated with physical activity (P=0.0001, r=268), modern clothing style (P=0.0001, r=600) and smoking (P=0.0001, r=273). Regional comparison of female patients demonstrated that significant differences exist in terms of educational status, clothing style, smoking, level of physical activity, calcium intake, and knowledge about osteoporosis. As a result, education has profound effects on awareness about osteoporosis and many aspects of human behavior, such as calcium intake, physical activity, clothing style and smoking. Significant disparities may be observed between the geographical regions of the same country. PMID:15197545

  5. The dizzy patient.

    PubMed

    Kutz, Joe Walter

    2010-09-01

    The dizzy patient often presents a challenge to the physician. The history is the most important component of the evaluation of the dizzy patient and often allows the cause of the dizziness to be categorized as peripheral or central. Peripheral causes include benign paroxysmal positional vertigo, Meniere's disease, and vestibular neuritis. Central causes include migraine-associated dizziness, postconcussion syndromes, cerebrovascular disease, and multiple sclerosis. Treatment depends on the cause of the dizziness and may include dietary modifications, diuretics, vestibular suppressants, vestibular rehabilitation, or surgical intervention. PMID:20736108

  6. Assessment of trauma patients.

    PubMed

    Parker, M; Magnusson, C

    2016-05-01

    Trauma is a major contributor to global mortality and morbidity with a notable difference between low income countries (LIC) and high to moderate income countries (HMIC). The modality of trauma differs globally; however, the most notable cause is pedestrian vs. vehicle and road traffic collision respectively. It is imperative that patients who have sustained a traumatic injury are managed in an appropriate and timely manner. Part 1 of the article will address the aetiology and demographic distribution of trauma globally and part 2 of the article will provide information about structured assessment and management of trauma patients. PMID:26655681

  7. Constructions of the patient in healthcare communications: six patient figures.

    PubMed

    Pors, Anja Svejgaard

    2016-04-11

    Purpose - The purpose of this paper is to examine how strategic, patient-centred communication plays a part in the discursive management of expectations posed to patients and healthcare organizations. Design/methodology/approach - The paper provides an analysis of four documents collected as part of an ethnographic case study regarding "The Perspective of the Patient" - a Danish Hospital's patient-centred communication programme. Mapping methods inspired by Grounded Theory are used to qualify the analysis. Findings - The paper shows that strategic patient-centred communication addresses both a care-oriented approach to the patient and deploys market perceptions of patients. Market and care is seen as co-existing organizing modes that entail expectations to the patient. In the communication programme the patient is constructed in six information-seeking patient figures: affective patient; target group patient; citizen with rights; patient as a competent resource; user as active partner; and consumer. As a result, the patient-centred communication programme renders the patient as a flexible figure able to fit organizational demands of both care orientation and market concerns. Originality/value - This study contributes to qualitative research in organizational health communication by combining two subfields - patient-centredness and health communication - in an empirical study of how market and care are intertwined in a patient-centred communication programme. The argument goes beyond the prevalent prescriptive approaches to patient-centredness and healthcare communication, instead providing a critical analytical perspective on strategic communication and patient-centredness and showing how expectations are posed to both patient and organization. PMID:27052626

  8. The physician-patient relationship: a patient-physician's view.

    PubMed

    Ennis, Jeffrey H

    1990-12-01

    The physician-patient relationship, like any human relationship, blends two types of interactions described by philosopher Martin Buber. In an "I-It" interaction, the physician objectifies the patient and his or her problem; in an "I-Thou" interaction, the physician perceives the patient as an emotional being. My encounters with medical practitioners as a patient with brachial neuritis and Guillain-Barré syndrome illustrate these forms of the physician-patient relationship. PMID:11651130

  9. Patient Participation: Current Knowledge and Applicability to Patient Safety

    PubMed Central

    Longtin, Yves; Sax, Hugo; Leape, Lucian L.; Sheridan, Susan E.; Donaldson, Liam; Pittet, Didier

    2010-01-01

    Patient participation is increasingly recognized as a key component in the redesign of health care processes and is advocated as a means to improve patient safety. The concept has been successfully applied to various areas of patient care, such as decision making and the management of chronic diseases. We review the origins of patient participation, discuss the published evidence on its efficacy, and summarize the factors influencing its implementation. Patient-related factors, such as acceptance of the new patient role, lack of medical knowledge, lack of confidence, comorbidity, and various sociodemographic parameters, all affect willingness to participate in the health care process. Among health care workers, the acceptance and promotion of patient participation are influenced by other issues, including the desire to maintain control, lack of time, personal beliefs, type of illness, and training in patient-caregiver relationships. Social status, specialty, ethnic origin, and the stakes involved also influence patient and health care worker acceptance. The London Declaration, endorsed by the World Health Organization World Alliance for Patient Safety, calls for a greater role for patients to improve the safety of health care worldwide. Patient participation in hand hygiene promotion among staff to prevent health care—associated infection is discussed as an illustrative example. A conceptual model including key factors that influence participation and invite patients to contribute to error prevention is proposed. Further research is essential to establish key determinants for the success of patient participation in reducing medical errors and in improving patient safety. PMID:20042562

  10. Dermatoses in geriatric patients.

    PubMed

    Kelly, R

    1977-01-01

    Geriatric patients, though they may present many of the dermatological problems occurring throughout adult life, show a greater incidence of the degenerative skin changes consequent on sun exposure in the form of collagen degeneration, solar keratoses, keratoacanthoma and skin cancer. The benign, precancerous and malignant skin tumours, the various types of eczema, senile pruritus and certain other conditions will be discussed. PMID:140650

  11. The Adolescent Patient.

    ERIC Educational Resources Information Center

    Daniel, William A., Jr.

    Written to orient the physician and paramedical personnel to the adolescent patient, the book provides information concerning the changes of adolescence, and age-related problems and illnesses. Part 1 discusses the essence of adolescence by describing physical, mental, and emotional growth and development. Part 2, the major section, consists of 21

  12. Thromboprophylaxis for trauma patients

    PubMed Central

    Lozano, Luis Manuel Barrera; Perel, Pablo; Ker, Katharine; Cirocchi, Roberto; Farinella, Eriberto; Morales, Carlos Hernando

    2014-01-01

    This is the protocol for a review and there is no abstract. The objectives are as follows: To assess the effects of thromboprophylaxis in trauma patients on mortality and incidence of DVT and PE. To compare the effects of different thromboprophylaxis interventions and their relative effects according to the type of trauma. PMID:25267908

  13. A Patient's Best Friend.

    ERIC Educational Resources Information Center

    Haggard, Ann

    1985-01-01

    The author describes the development of a pet therapy program at a rehabilitation unit of a hospital. She discusses documentation of the success of pet therapy programs, picking out the right pets, responsibilities of caring for the pet, training the pet, patient response, and program policies. (CT)

  14. [Complications of patient positioning].

    PubMed

    Staender, S

    1995-01-01

    Complications related to wrong or improper perioperative positioning are preventable events. Nevertheless patients may suffer from these complications much more than from the original pathology. In a short review a focus is set on the prone and supine position. Finally remarks for prevention are added. PMID:8590298

  15. The Adolescent Patient.

    ERIC Educational Resources Information Center

    Daniel, William A., Jr.

    Written to orient the physician and paramedical personnel to the adolescent patient, the book provides information concerning the changes of adolescence, and age-related problems and illnesses. Part 1 discusses the essence of adolescence by describing physical, mental, and emotional growth and development. Part 2, the major section, consists of 21…

  16. Teleophthalmology: improving patient outcomes?

    PubMed

    Sreelatha, Omana Kesary; Ramesh, Sathyamangalam VenkataSubbu

    2016-01-01

    Teleophthalmology is gaining importance as an effective eye care delivery modality worldwide. In many developing countries, teleophthalmology is being utilized to provide quality eye care to the underserved urban population and the unserved remote rural population. Over the years, technological innovations have led to improvement in evidence and teleophthalmology has evolved from a research tool to a clinical tool. The majority of the current teleophthalmology services concentrate on patient screening and appropriate referral to experts. Specialty care using teleophthalmology services for the pediatric group includes screening as well as providing timely care for retinopathy of prematurity (ROP). Among geriatric eye diseases, specialty teleophthalmology care is focused toward screening and referral for diabetic retinopathy (DR), glaucoma, age-related macular degeneration (ARMD), and other sight-threatening conditions. Comprehensive vision screening and refractive error services are generally covered as part of most of the teleophthalmology methods. Over the past decades, outcome assessment of health care system includes patients' assessments on their health, care, and services they receive. Outcomes, by and large, remain the ultimate validators of the effectiveness and quality of medical care. Teleophthalmology produces the same desired clinical outcome as the traditional system. Remote portals allow specialists to provide care over a larger region, thereby improving health outcomes and increasing accessibility of specialty care to a larger population. A high satisfaction level and acceptance is reported in the majority of the studies because of increased accessibility and reduced traveling cost and time. Considering the improved quality of patient care and patient satisfaction reported for these telemedicine services, this review explores how teleophthalmology helps to improve patient outcomes. PMID:26929592

  17. [Patients' University, illness and learning].

    PubMed

    Tourette-Turgis, Catherine

    2015-10-01

    The Patients' University, a pilot project at the Université Pierre et Marie Curie, in Paris, enables patients-experts to follow a degree program in patient therapeutic education (University Diploma and Master). Recently, graduate patients and patients directly concerned proposed to co-create a new university certificate for treatment pathway coordinators for breast cancer, rounding out the 120-hour university certificate program on healthcare democracy and meeting the recommendations of the new cancer plan. PMID:26455618

  18. The Physician-Patient Relationship

    PubMed Central

    Ennis, Jeffrey H.

    1990-01-01

    The physician-patient relationship, like any human relationship, blends two types of interactions described by philosopher Martin Buber. In an “I-It” interaction, the physician objectifies the patient and his or her problem; in an “I-Thou” interaction, the physician perceives the patient as an emotional being. My encounters with medical practitioners as a patient with brachial neuritis and Guillain-Barré syndrome illustrate these forms of the physician-patient relationship. Imagesp2216-a PMID:11651130

  19. Lysosomal Multienzyme Complex: Pros and Cons of Working Together

    PubMed Central

    Bonten, Erik J.; Annunziata, Ida; d’Azzo, Alessandra

    2014-01-01

    The ubiquitous distribution of lysosomes and their heterogeneous protein composition reflects the versatility of these organelles in maintaining cell homeostasis and their importance in tissue differentiation and remodeling. In lysosomes, the degradation of complex, macromolecular substrates requires the synergistic action of multiple hydrolases that usually work in a stepwise fashion. This catalytic machinery explains the existence of lysosomal enzyme complexes that can be dynamically assembled and disassembled to efficiently and quickly adapt to the pool of substrates to be processed or degraded, adding extra tiers to the regulation of the individual protein components. An example of such a complex is the one composed of three hydrolases that are ubiquitously but differentially expressed: the serine carboxypeptidase, Protective Protein/Cathepsin A (PPCA), the sialidase, Neuraminidase-1 (NEU1), and the glycosidase β-Galactosidase (β-GAL). Next to this ‘core’ complex, the existence of sub-complexes, that may contain additional components, and function at the cell surface or extracellularly, suggests as yet unexplored functions of these enzymes. Here we review how studies of basic biological processes in the mouse models of three lysosomal storage disorders, galactosialidosis, sialidosis, and GM1-gangliosidosis, revealed new and unexpected roles for the three respective affected enzymes, Ppca, Neu1 and β-Gal, that go beyond their canonical degradative activities. These findings have broadened our perspective on their functions and may pave the way for the development of new therapies for these lysosomal storage disorders. PMID:24337808

  20. Metabolic cardiomyopathies

    PubMed Central

    Guertl, Barbara; Noehammer, Christa; Hoefler, Gerald

    2000-01-01

    The energy needed by cardiac muscle to maintain proper function is supplied by adenosine Ariphosphate primarily (ATP) production through breakdown of fatty acids. Metabolic cardiomyopathies can be caused by disturbances in metabolism, for example diabetes mellitus, hypertrophy and heart failure or alcoholic cardiomyopathy. Deficiency in enzymes of the mitochondrial β-oxidation show a varying degree of cardiac manifestation. Aberrations of mitochondrial DNA lead to a wide variety of cardiac disorders, without any obvious correlation between genotype and phenotype. A completely different pathogenetic model comprises cardiac manifestation of systemic metabolic diseases caused by deficiencies of various enzymes in a variety of metabolic pathways. Examples of these disorders are glycogen storage diseases (e.g. glycogenosis type II and III), lysosomal storage diseases (e.g. Niemann-Pick disease, Gaucher disease, I-cell disease, various types of mucopolysaccharidoses, GM1 gangliosidosis, galactosialidosis, carbohydrate–deficient glycoprotein syndromes and Sandhoff's disease). There are some systemic diseases which can also affect the heart, for example triosephosphate isomerase deficiency, hereditary haemochromatosis, CD 36 defect or propionic acidaemia. PMID:11298185

  1. [Infections in immunosuppressed patients].

    PubMed

    Marcos, Mara Angeles; Alvarez-Martnez, Miriam J; Niub, Jordi; Pumarola, Toms

    2008-07-01

    Molecular biology techniques represent a major advance in the microbiologic diagnosis of infectious diseases, since these methods are able to detect etiological microorganisms with high sensitivity. Moreover, these procedures can also establish prognostic and therapeutic efficacy markers with a sufficiently short turnaround time for the results to have a real impact on the clinical management of immunosuppressed patients. However, these techniques still have substantial limitations that should be solved in the near future: lack of standardization, inter- and intra-assay variability, the difficulty of comparing results among different laboratories and low positive predictive value, due to their high sensitivity, leading to problems in the interpretation of results. The present article reviews the usefulness of molecular biology techniques in the diagnosis and clinical management of infectious diseases caused by human cytomegalovirus, Epstein-Barr virus, human herpes viruses 6 and 7, JC and BK viruses, Toxoplasma gondii and Pneumocystis jiroveci in immunosuppressed patients. PMID:19195448

  2. Patient population controls.

    PubMed

    Carey, R Neill

    2013-03-01

    Quality control (QC) procedures incorporating patient means, or average of normals (AoN) algorithms, have been used in hematology laboratories and large reference laboratories for decades to monitor analytical processes during the periods between the testing of reference sample QC materials. With the advent of middleware that includes AoN capability, these QC procedures are now available to many more laboratories, including medium-sized hospital laboratories. AoN procedures can improve the control of tests that have low "sigma-metrics," such as electrolytes, where relatively low numbers of patient results can be averaged to provide a high probability of detecting medically significant errors. QC of nearly one-third of the tests whose AoN capabilities have been studied would possibly benefit from AoN procedures in medium-sized laboratories. To obtain satisfactory performance, laboratories must tailor the applications of AoN procedures to their particular volumes and test characteristics. PMID:23331734

  3. Melanoma in Black Patients

    PubMed Central

    Smith, Robert J.

    1982-01-01

    Melanoma in black patients is uncommon but not rare. This paper reports six cases seen in one general surgeon's practice in Arkansas during a 14-year period. A review of the current literature regarding melanoma in blacks is given. Characteristically, melanoma in blacks is found on the soles of the feet, palms of the hands, or mucous membranes. The tumor has a deadly potential unless it is treated at an early stage; four of the patients reported have died of metastatic disease. A public health program is needed to make physicians and the public aware of the incidence and location of this tumor. ImagesFigure 1Figure 2Figure 3Figure 4 PMID:7120473

  4. Teleophthalmology: improving patient outcomes?

    PubMed Central

    Sreelatha, Omana Kesary; Ramesh, Sathyamangalam VenkataSubbu

    2016-01-01

    Teleophthalmology is gaining importance as an effective eye care delivery modality worldwide. In many developing countries, teleophthalmology is being utilized to provide quality eye care to the underserved urban population and the unserved remote rural population. Over the years, technological innovations have led to improvement in evidence and teleophthalmology has evolved from a research tool to a clinical tool. The majority of the current teleophthalmology services concentrate on patient screening and appropriate referral to experts. Specialty care using teleophthalmology services for the pediatric group includes screening as well as providing timely care for retinopathy of prematurity (ROP). Among geriatric eye diseases, specialty teleophthalmology care is focused toward screening and referral for diabetic retinopathy (DR), glaucoma, age-related macular degeneration (ARMD), and other sight-threatening conditions. Comprehensive vision screening and refractive error services are generally covered as part of most of the teleophthalmology methods. Over the past decades, outcome assessment of health care system includes patients’ assessments on their health, care, and services they receive. Outcomes, by and large, remain the ultimate validators of the effectiveness and quality of medical care. Teleophthalmology produces the same desired clinical outcome as the traditional system. Remote portals allow specialists to provide care over a larger region, thereby improving health outcomes and increasing accessibility of specialty care to a larger population. A high satisfaction level and acceptance is reported in the majority of the studies because of increased accessibility and reduced traveling cost and time. Considering the improved quality of patient care and patient satisfaction reported for these telemedicine services, this review explores how teleophthalmology helps to improve patient outcomes. PMID:26929592

  5. [Sport for pacemaker patients].

    PubMed

    Israel, C W

    2012-06-01

    Sport activity is an important issue in many patients with a pacemaker either because they performed sport activities before pacemaker implantation to reduce the cardiovascular risk or to improve the course of an underlying cardiovascular disease (e.g. coronary artery disease, heart failure) by sports. Compared to patients with an implantable cardioverter defibrillator (ICD) the risks from underlying cardiovascular disease (e.g. ischemia, heart failure), arrhythmia, lead dysfunction or inappropriate therapy are less important or absent. Sport is contraindicated in dyspnea at rest, acute heart failure, new complex arrhythmia, acute myocarditis and acute myocardial infarction, valvular disease with indications for intervention and surgery and comorbidities which prevent physical activity. Patients with underlying cardiovascular disease (including hypertension) should preferably perform types and levels of physical activity that are aerobic (with dynamic exercise) such as running, swimming, cycling instead of sport with high anaerobic demands and high muscular workload. In heart failure, studies demonstrated advantages of isometric sport that increases the amount of muscle, thereby preventing cardiac cachexia. Sport with a risk of blows to the chest or physical contact (e.g. boxing, rugby, martial arts) should be avoided. Implantation, programming and follow-up should respect specific precautions to allow optimal physical activity with a pacemaker including implantation of bipolar leads on the side contralateral to the dominant hand, individual programming of the upper sensor and tracking rate and regular exercise testing. PMID:22854824

  6. Nutrition in cancer patients.

    PubMed

    Dintinjana, Renata Dobrila; Redzović, Arnela; Cubranić, Aleksandar; Dintinjana, Marijan; Vanis, Nenad

    2014-12-01

    Cachexia is defined as an unintended loss of stable weight exceeding 10%. Patients with advanced cachexia express anorexia, early satiety, severe weight loss, weakness, anemia, and edema. Anorexia represents the result of a failure of the usual appetite signals whereas cachexia is the debilitating state of involuntary weight loss. This syndrome, referred to as the cancer anorexia-cachexia syndrome, (CACS) and usually consists of a combination of anorexia, tissue wasting, malnutrition, weight loss and loss of compensatory increase in feeding. CACS represents the result of a complex interaction between cancer growth and host response and is associated with a poor response to chemotherapy and with an increase in drug-related toxicity. In advanced cachexia (mostly in metastatic cancer and terminally disease) any interventions with nutritional suplements are ineffective. Therefore, nutritional support in the reversion of tumor cachexia and in the importance of maintaining patient weight, muscle mass, quality of life, has the exceptional importance, becouse good nutritional status of patients leads to the possibility of more aggressive and longer treatment and thus to longer survival. PMID:25842774

  7. Nutrition in cancer patients.

    TOXLINE Toxicology Bibliographic Information

    Dintinjana RD; Redzović A; Cubranić A; Dintinjana M; Vanis N

    2014-12-01

    Cachexia is defined as an unintended loss of stable weight exceeding 10%. Patients with advanced cachexia express anorexia, early satiety, severe weight loss, weakness, anemia, and edema. Anorexia represents the result of a failure of the usual appetite signals whereas cachexia is the debilitating state of involuntary weight loss. This syndrome, referred to as the cancer anorexia-cachexia syndrome, (CACS) and usually consists of a combination of anorexia, tissue wasting, malnutrition, weight loss and loss of compensatory increase in feeding. CACS represents the result of a complex interaction between cancer growth and host response and is associated with a poor response to chemotherapy and with an increase in drug-related toxicity. In advanced cachexia (mostly in metastatic cancer and terminally disease) any interventions with nutritional suplements are ineffective. Therefore, nutritional support in the reversion of tumor cachexia and in the importance of maintaining patient weight, muscle mass, quality of life, has the exceptional importance, becouse good nutritional status of patients leads to the possibility of more aggressive and longer treatment and thus to longer survival.

  8. COPD: the patient perspective

    PubMed Central

    Jones, Paul W; Watz, Henrik; Wouters, Emiel FM; Cazzola, Mario

    2016-01-01

    Chronic obstructive pulmonary disease (COPD) is a highly prevalent disease characterized by nonreversible airway obstruction. Well-characterized symptoms such as exertional dyspnea and fatigue have a negative impact on patients’ quality of life (QoL) and restrict physical activity in daily life. The impact of COPD symptoms on QoL is often underestimated; for example, 36% of patients who describe their symptoms as being mild-to-moderate also admit to being too breathless to leave the house. Additionally, early morning and nighttime symptoms are a particular problem. Methods are available to allow clinicians to accurately assess COPD symptoms, including patient questionnaires. Integrated approaches to COPD management, particularly pulmonary rehabilitation, are effective strategies for addressing symptoms, improving exercise capacity and, potentially, also increasing physical activity. Inhaled bronchodilators continue to be the mainstay of drug therapy in COPD, where options can be tailored to meet patients’ needs with careful selection of the inhaled medication and the device used for its delivery. Overall, an integrated approach to disease management should be considered for improving QoL and subsequent patient outcomes in COPD. PMID:26937186

  9. A novel ganglioside, 9-O-acetyl GD1b, is recognized by serum antibodies in Guillain-Barré syndrome.

    PubMed

    Hitoshi, S; Kusunoki, S; Kon, K; Chiba, A; Waki, H; Ando, S; Kanazawa, I

    1996-05-01

    A hitherto undescribed ganglioside was detected in a crude ganglioside fraction of bovine brain using an IgM M-protein binding to Gal beta 1, 3GalNAc residue. We purified and identified it as 9-O-acetyl GD1b based on results of alkali treatment that yielded GD1b and results of fast atom bombardment-mass and gas chromatography-mass spectrometries. 9-O-acetyl GD1b was also found to be present in human peripheral nerve tissue. The reactivities of the serum antibodies from patients with Guillain-Barré syndrome to 9-O-acetyl GD1b, GD1b, and GM1 were determined by ELISA and TLC immunostaining. Nineteen of 85 serum samples from Guillain-Barré syndrome patients had antibodies that bound to 9-O-acetyl GD1b: 14 of the positive samples also reacted with GM1 and GD1b, three reacted with GM1 but not with GD1b, one with GD1b but not with GM1, and one with neither GM1 nor GD1b. These results show that a subset of patients with Guillain-Barré syndrome had antibodies that react with 9-O-acetyl GD1b; therefore, this ganglioside can serve as a target antigen against the antibodies present in Guillain-Barré syndrome. PMID:8964919

  10. The challenge of patient adherence

    PubMed Central

    Martin, Leslie R; Williams, Summer L; Haskard, Kelly B; DiMatteo, M Robin

    2005-01-01

    Quality healthcare outcomes depend upon patients' adherence to recommended treatment regimens. Patient nonadherence can be a pervasive threat to health and wellbeing and carry an appreciable economic burden as well. In some disease conditions, more than 40% of patients sustain significant risks by misunderstanding, forgetting, or ignoring healthcare advice. While no single intervention strategy can improve the adherence of all patients, decades of research studies agree that successful attempts to improve patient adherence depend upon a set of key factors. These include realistic assessment of patients' knowledge and understanding of the regimen, clear and effective communication between health professionals and their patients, and the nurturance of trust in the therapeutic relationship. Patients must be given the opportunity to tell the story of their unique illness experiences. Knowing the patient as a person allows the health professional to understand elements that are crucial to the patient's adherence: beliefs, attitudes, subjective norms, cultural context, social supports, and emotional health challenges, particularly depression. Physician–patient partnerships are essential when choosing amongst various therapeutic options to maximize adherence. Mutual collaboration fosters greater patient satisfaction, reduces the risks of nonadherence, and improves patients' healthcare outcomes. PMID:18360559

  11. Patterns of Physician-Patient Communication Associated with Patient Satisfaction.

    ERIC Educational Resources Information Center

    Williams, M. Lee; Clampitt, Phillip G.

    Using data drawn from ten initial physician/patient interviews, an original category system was employed to analyze patterns of physician/patient communication. Static analysis, interaction analysis, and Markov chain analysis were used to discover the underlying communication patterns associated with patient satisfaction. Results revealed that…

  12. Copying referral letters to patients: the views of patients, patient representatives and doctors.

    PubMed

    White, Philip; Singleton, Andrew; Jones, Roger

    2004-10-01

    To discover the views of patients, patient representatives and doctors on copying referral letters to patients. A three-part study: an analysis of 50 GP referral letters against a standard template; 35 patient interviews using a semi-structured questionnaire in outpatient waiting rooms; 3 focus groups of patients, patient representatives and doctors. There was general agreement that copying referral letters to patients could improve information and decision sharing with patients. Copying referral letters could provide an opportunity for patients to correct mistakes, prepare for their appointments and have a personal record that they could keep and show to others. However, there were concerns about letter content, particularly medical terminology, character judgements and "sensitive" patient information. It was also recognised that providing more information to patients could increase patient anxiety. The style and content of some referral letters may need to change. This is particularly relevant where certain types of information included in referral letters could cause distress for patients or influence the time that patients have to wait for their outpatient appointments. PMID:15476995

  13. Help patients cope with chemo.

    PubMed

    Nagel, Teresa

    2004-10-01

    Chemotherapy is keeping more and more patients alive, but its side effects can be debilitating. Appropriate nursing care and education can help cancer patients overcome the challenges of this vital treatment. PMID:15552377

  14. Patient Safety Threat - Syringe Reuse

    MedlinePlus

    ... About CDC.gov . Injection Safety Share Compartir A Patient Safety Threat – Syringe Reuse Important Information! Please read ... due to syringe reuse by your healthcare provider. Patients need to be aware of a very serious ...

  15. Restoring Speech to Tracheostomy Patients.

    PubMed

    Morris, Linda L; Bedon, Ana M; McIntosh, Erik; Whitmer, Andrea

    2015-12-01

    Tracheostomies may be established as part of an acute or chronic illness, and intensive care nurses can take an active role in helping restore speech in patients with tracheostomies, with focused nursing assessments and interventions. Several different methods are used to restore speech, whether a patient is spontaneously breathing, ventilator dependent, or using intermittent mechanical ventilation. Restoring vocal communication allows patients to fully express themselves and their needs, enhancing patient satisfaction and quality of life. PMID:26628542

  16. Overcoming barriers to patient safety.

    PubMed

    Kalisch, Beatrice J; Aebersold, Michelle

    2006-01-01

    Creating a culture of patient safety is a critical goal of all patient care unit staff. An analysis of the key barriers to patient safety on a typical inpatient unit in an acute care hospital (unclear unit values), the fear of punishment for errors, the lack of systematic analysis of mistakes, the complexity of the nurses' work, and inadequate teamwork are presented. Nine practices to overcome these barriers and achieve patient safety are discussed. PMID:16786829

  17. The patient-friendly practice.

    PubMed

    Dooley, Sharon Kay

    2006-01-01

    In today's medical marketplace, patients see themselves as consumers of healthcare with certain customer-service expectations. The medical practice that is indifferent or resistant to these changes is at risk. Having a good understanding of patient-friendly changes can help a practice survive in a changing environment. A patient-friendly office will continue to meet the needs of the patient by adopting this new practice style. PMID:16833071

  18. The physician-patient relationship revisited: the patient's view.

    PubMed

    Schneider, Udo; Ulrich, Volker

    2008-12-01

    The importance of the physician-patient relationship for the health care market is beyond controversy. Recent work emphasizes a two-sided asymmetric information relationship between physician and patient. In contrast to most work looking only at the physician's perspective, our paper concentrates on the patient's view. Estimation results support the hypotheses that physician consultation and health relevant behavior are not stochastically independent. In the recursive bivariate probit model, patient's health relevant behavior has a significant influence on the probability of a physician visit. This means that health care demand and not only the contact decision is determined by both, patient and physician. PMID:18597172

  19. Selection of patients

    PubMed Central

    Leenen, H J J

    1982-01-01

    The author joins the discussion on selection of patients in the face of life-saving resources initiated in the Journal by Parsons and Lock, Mooney and the editorial in the December 1980 issue. In this article several selection systems are discussed. The author is in favour of a `criteria-system'. The criteria for such a system are elaborated. On the basis of a sequence of values a sequence of criteria is proposed. Attention is also given to the procedural aspects. PMID:7069733

  20. [Patients with sepsis].

    PubMed

    Oppert, M

    2016-05-01

    Sepsis is still the leading cause of mortality in noncardiac intensive care units. The new definition of sepsis emphasizes the importance of organ dysfunction. The Sepsis-related Organ Failure Assessment (SOFA) score is an indicator for organ dysfunction. The diagnosis of sepsis is for the most part made on clinical parameters with an altered mental status being a very sensitive indicator. Microbiological work-up is essential and two sets of blood cultures are the recommended minimum. Management includes prompt initiation of adequate antibiotic treatment and swift fluid resuscitation. Overinfusion is to be avoided as this itself can have a negative impact on patient outcome. PMID:27160262

  1. Dysphagia in stroke patients

    PubMed Central

    Singh, S; Hamdy, S

    2006-01-01

    Swallowing musculature is asymmetrically represented in both motor cortices. Stroke affecting the hemisphere with the dominant swallowing projection results in dysphagia and clinical recovery has been correlated with compensatory changes in the previously non‐dominant, unaffected hemisphere. This asymmetric bilaterality may explain why up to half of stroke patients are dysphagic and why many will regain a safe swallow over a comparatively short period. Despite this propensity for recovery, dysphagia carries a sevenfold increased risk of aspiration pneumonia and is an independent predictor of mortality. The identification, clinical course, pathophysiology, and treatment of dysphagia after stroke are discussed in this review. PMID:16754707

  2. Patient perception of wound photography.

    PubMed

    Wang, Sheila C; Anderson, John Ae; Jones, Duncan Vb; Evans, Robyn

    2016-06-01

    The objectives of this study were to provide an assessment of photographic documentation of the wound from the patients' perspective and to evaluate whether this could improve patients' understanding of and involvement in their wound care. Our results revealed that most patients visiting the wound care clinic have difficult-to-see wounds (86%). Only 20% of patients monitor their wounds and instead rely on clinic or nurse visits to track the healing progress. There was a significant association between patients' ability to see their wound and their subsequent memory of the wound's appearance. This was especially true for patients who had recently begun visiting the wound care clinic. This relationship was not present in patients who had visited the clinic for 3  or more years. Patients reported that the inability to see their wounds resulted in feeling a loss of autonomy. The majority of patients reported that photographing their wounds would help them to track the wound progress (81%) and would afford them more involvement in their own care (58%). This study provides a current representation of wound photography from the patients' perspective and reveals that it can motivate patients to become more involved in the management of their wounds - particularly for patients with difficult-to-see wounds. PMID:24872018

  3. [Organizing patient education in cardiology].

    PubMed

    Fischer, Eric; Thieffry, Eliane

    2014-02-01

    A central element of the care management of patients with heart failure, therapeutic patient education mobilises caregivers into forming a multi-disciplinary team. In this article, a hospital team shares the different stages in the construction and implementation of a programme for use with hospitalised patients and in consultations. To do this, the nurses undertook training to acquire new educational skills. PMID:24654334

  4. Patient satisfaction with physician-patient communication during telemedicine.

    PubMed

    Agha, Zia; Schapira, Ralph M; Laud, Purushottam W; McNutt, Gail; Roter, Debra L

    2009-11-01

    The quality of physician-patient communication is a critical factor in treatment outcomes and patient satisfaction with care. To date, few studies have specifically conducted an in-depth evaluation of the effect of telemedicine (TM) on physician-patient communication in a medical setting. Our objective was to determine whether physical separation and technology used during TM have a negative effect on physician-patient communication. In this noninferiority randomized clinical trial, patients were randomized to receive a single consultation with one of 9 physicians, either in person (IP) or via TM. Patients (n = 221) were recruited from pulmonary, endocrine, and rheumatology clinics at a Midwestern Veterans Administration hospital. Physician-patient communication was measured using a validated self-report questionnaire consisting of 33 items measuring satisfaction with visit convenience and physician's patient-centered communication, clinical competence, and interpersonal skills. Satisfaction for physician's patient-centered communication was similar for both consultation types (TM = 3.76 versus IP = 3.61), and noninferiority of TM was confirmed (noninferiority t-test p = 0.002). Patient satisfaction with physician's clinical competence (TM = 4.63 versus IP = 4.52) and physician's interpersonal skills (TM = 4.79 versus IP = 4.74) were similar, and noninferiority of TM was confirmed (noninferiority t-test p = 0.006 and p = 0.04, respectively). Patients reported greater satisfaction with convenience for TM as compared to IP consultations (TM = 4.41 versus IP = 2.37, noninferiority t-test p < 0.001). Patients were equally satisfied with physician's ability to develop rapport, use shared decision making, and promote patient-centered communication during TM and IP consultations. Our data suggest that, despite physical separation, physician-patient communication during TM is not inferior to communication during IP consultations. PMID:19919189

  5. Informed Consent for AAA Repair: The Patients Perspective

    PubMed Central

    Berman, Loren; Curry, Leslie; Gusberg, Richard; Dardik, Alan; Fraenkel, Liana

    2009-01-01

    BACKGROUND Although information about risks, benefits and alternatives to intervention is central to ensuring adequate informed consent, patients are often not well-informed about potential adverse outcomes when they are considering whether to have surgery. Whether or not to undergo surgery for abdominal aortic aneurysm (AAA), and whether to have open or endovascular repair (EVAR), is a complex decision that relies heavily on patient preferences, and yet little is known about the patient perspective on informed consent in this context. Understanding patients views on their decision-making processes and the quality of surgeon-patient communication could inform improvements in informed consent for AAA repair. METHODS We conducted in-depth interviews with AAA patients (n=20) who underwent open AAA repair, endovascular repair, or declined surgery. Data were independently transcribed and analyzed by a team of individuals with diverse backgrounds, using the constant comparative method of analysis and systematic coding procedures. RESULTS We identified four central themes characterizing patients experiences with informed consent for AAA repair: 1) some patients perceived that there was no choice regarding whether or not to have surgery; 2) some patients did not feel adequately informed prior to making a decision; 3) patients differed in the scope and content of information they desired during informed consent; and 4) trust in the surgeon had an impact on the informed consent process. CONCLUSION Our research highlights the limitations of the informed consent encounter in the current clinical context, and points to several ways in which informed consent could be improved. Adapting the informed consent encounter to incorporate the patients perspective is critical in order to ensure that the decision regarding AAA repair is consistent with the patients informed preference. PMID:18572357

  6. Patients' perspectives on pain.

    PubMed

    Norrbrink, Cecilia; Löfgren, Monika; Hunter, Judith P; Ellis, Jaqueline

    2012-01-01

    Nociceptive and neuropathic pain (NP) are common consequences following spinal cord injury (SCI), with large impact on sleep, mood, work, and quality of life. NP affects 40% to 50% of individuals with SCI and is sometimes considered the major problem following SCI. Current treatment recommendations for SCI-NP primarily focus on pharmacological strategies suggesting the use of anticonvulsant and antidepressant drugs, followed by tramadol and opioid medications. Unfortunately, these are only partly successful in relieving pain. Qualitative studies report that individuals with SCI-related long-lasting pain seek alternatives to medication due to the limited efficacy, unwanted side effects, and perceived risk of dependency. They spend time and money searching for additional treatments. Many have learned coping strategies on their own, including various forms of warmth, relaxation, massage, stretching, distraction, and physical activity. Studies indicate that many individuals with SCI are dissatisfied with their pain management and with the information given to them about their pain, and they want to know more about causes and strategies to manage pain. They express a desire to improve communication with their physicians and learn about reliable alternative sources for obtaining information about their pain and pain management. The discrepancy between treatment algorithms and patient expectations is significant. Clinicians will benefit from hearing the patient´s voice. PMID:23459087

  7. The 'Patient experience' revolution.

    PubMed

    Hooten, Doug; Zavadsky, Matt

    2014-02-01

    We're arguably at the most pivotal time in our young profession. The ACA has provided EMS an unprecedented opportunity to become a part of the healthcare system, a move that many of us have dreamed about for decades. We need to pay attention to the changing dynamics of the environment in which we operate. The factors that currently impact hospitals, doctors and other healthcare providers will also impact us sooner than we think. Take the time to help shape our future and how we participate in this new healthcare system. It's time to focus on the patient and the patient's experience with our service. Wayne Gretzky said two important things during an interview when he was asked what makes him such a great hockey player. One was, "You miss 100% of the shots you don't take." The other was, "A good hockey player plays where the puck is. A great hockey player plays where the puck is going to be. I skate to where the puck is going to be, not where it has been." Our advice to you is to go ahead, take the shot, get ahead of the other team and focus on improved customer satisfaction sooner rather than later. PMID:24660359

  8. Caring for Latino patients.

    PubMed

    Juckett, Gregory

    2013-01-01

    Latinos comprise nearly 16 percent of the U.S. population, and this proportion is anticipated to increase to 30 percent by 2050. Latinos are a diverse ethnic group that includes many different cultures, races, and nationalities. Barriers to care have resulted in striking disparities in quality of health care for these patients. These barriers include language, lack of insurance, different cultural beliefs, and in some cases, illegal immigration status, mistrust, and illiteracy. The National Standards for Culturally and Linguistically Appropriate Services address these concerns with recommendations for culturally competent care, language services, and organizational support. Latinos have disproportionately higher rates of obesity and diabetes mellitus. Other health problems include stress, neurocysticercosis, and tuberculosis. It is important to explore the use of alternative therapies and belief in traditional folk illnesses, recognizing that health beliefs are dependent on education, socioeconomic status, and degree of acculturation. Many-but not all-folk and herbal treatments can be safely accommodated with conventional therapy. Physicians must be sensitive to Latino cultural values of simpatia (kindness), personalismo (relationship), respeto (respect), and modestia (modesty). The LEARN technique can facilitate cross-cultural interviews. Some cultural barriers may be overcome by using the "teach back" technique to ensure that directions are correctly understood and by creating a welcoming health care environment for Latino patients. PMID:23317025

  9. [Anxiety in epileptic patients].

    PubMed

    Kotov, A S

    2013-01-01

    We examined 182 patients with epilepsy using the evaluation of anamnesis, clinical and neurological examination, routine EEG and/or video-EEG-monitoring, MRI of the brain. The period of observation was 1-11 years (on average 2 years). Anxiety and depression symptoms were screened with the Hospital anxiety and depression scale (HADS). Health-related quality of life was examined with the Quality of Life in Epilepsy Inventory (QOLIE-31, version 1). The diagnosis was confirmed in 166 cases and nonepileptic seizures were diagnosed in 16 cases. Subclinical anxiety (8-10 HADS scores) was identified in 23 (12.6%), clinical anxiety (>10 scores) - in 47 (25.8%), subclinical depression (8-10 scores) - in 25 (13.7%) and clinical depression (>10 scores) - in 23 (12.6%) cases. Comorbidity of anxiety and depression was noted in 33 (18.1%) patients. The high level of anxiety was correlated with the young age and short duration of epilepsy. Anxiety (r=0.7; p<0.05) and depression (r=0.63; p<0.05) were significantly correlated with the poor quality of life. PMID:23739454

  10. Patient Zero”:

    PubMed Central

    McKay, Richard A.

    2014-01-01

    Summary This article contextualizes the production and reception of And the Band Played On, Randy Shilts’s popular history of the initial recognition of the American AIDS epidemic. Published over twenty-five years ago, the book and its most notorious character, “Patient Zero,” are in particular need of a critical historical treatment. The article presents a more balanced consideration—a “patient’s view”—of Gaétan Dugas’s experience of the early years of AIDS. I oppose the assertion that Dugas, the so-called Patient Zero, ignored incontrovertible information about the condition and was intent on spreading his infection. Instead I argue that scientific ideas in 1982 and 1983 about AIDS and the transmissibility of a causative agent were later portrayed to be more self-evident than they were at the time. The article also traces how Shilts’s highly selective—and highly readable—characterization of Dugas rapidly became embedded in discussions about the need to criminalize the reckless transmission of HIV. PMID:24769806

  11. Preoperative Assessment of Geriatric Patients.

    PubMed

    Knittel, Justin G; Wildes, Troy S

    2016-03-01

    Geriatric patients are over-represented in hospitalizations, surgeries, and perioperative complications. Special consideration is required for this patient group in the perioperative period because of the prevalence of comorbid diseases, functional impairments, and other deficits. A comprehensive preoperative evaluation strategy is recommended to identify and address these issues. Systematic, multidomain assessments should be performed and paired with risk reduction efforts. A shared understanding of patient function and long-term health goals is also important for providing patient-centered care of the geriatric surgical patient. PMID:26927746

  12. [Falls in patients with dementia].

    PubMed

    Yamaguchi, Kiyoshi

    2008-11-01

    People with cognitive impairment are at about 2 to 3 times higher risk of falling compared with cognitively intact elderly. Incidence of falls among patients with Alzheimer's disease (AD) is high, nevertheless the clinical feature common in patients with mild to moderate AD is the absence of motor impairment. Recent studies suggest that the divided attention markedly impairs the ability of patients with AD to regulate the gait. Falls are particularly common in Dementia with Lewy bodies (DLB) patients and may aid diagnosis, and the falls are associated with parkinsonism and other unclear factors. Treatment studies evaluating fall reduction strategies in dementia patients are a priority. PMID:18974447

  13. Bibliotherapy in a patients' library.

    PubMed

    McDowell, D J

    1971-07-01

    This paper describes the involvement of patients in the Patients' Library at McLean Hospital, and the relationship between them and the librarian in library activities. The publication of a patients' magazine is discussed, with case histories of persons who had taken part in its production. The Patients' Librarian has a personal role in patient therapy, and accounts are given of various activities such as play-reading, poetry-reading, and the discussion of poems by established writers, with therapeutic aims in view. Actual clinical experiences are given. PMID:5146769

  14. Patient Perspectives on Biosimilar Insulin.

    PubMed

    Wilkins, Alasdair R; Venkat, Manu V; Brown, Adam S; Dong, Jessica P; Ran, Nina A; Hirsch, James S; Close, Kelly L

    2014-01-01

    Given that a new wave of biosimilar insulins will likely enter the market in coming years, it is important to understand patient perspectives on these biosimilars. A survey (N = 3214) conducted by the market research company dQ&A, which maintains a 10 000-patient panel of people with type 1 or type 2 diabetes in roughly equal measure, investigated these perspectives. The survey asked whether patients would switch to a hypothetical less expensive biosimilar insulin that was approved by their provider. Approximately 66% of respondents reported that they would "definitely" or "likely" use a biosimilar insulin, while 17% reported that they were "unlikely" to use or would "definitely not use" such a product. Type 2 diabetes patients demonstrated slightly more willingness to use biosimilars than type 1 diabetes patients. Common patient concerns included whether biosimilars would be as effective as reference products (~650 respondents), whether side effect profiles would deviate from those of reference products (~220 respondents), and the design of the delivery device (~50 respondents). While cost savings associated with biosimilar insulins could increase patient uptake, especially among patients without health insurance (some recent estimates suggest that biosimilars will come at a substantial discount), patients may still need assurance that a cheaper price tag is not necessarily associated with substandard quality. Overall, the dQ&A survey indicates that the majority of patients are willing to consider biosimilar insulins, but manufacturers will need to work proactively to address and assuage patient concerns regarding efficacy, safety, drug administration, and other factors. PMID:24876533

  15. [Treatment of patients with osteoarthritis].

    PubMed

    Vargas Negrín, Francisco; Medina Abellán, María D; Hermosa Hernán, Juan Carlos; de Felipe Medina, Ricardo

    2014-01-01

    The therapeutic management of patients with osteoarthritis aims to decrease pain and inflammation, improve physical function, and to apply safe and effective treatments. A patient-centered approach implies the active participation of the patient in the design of the treatment plan and in timely and informed decision-making at all stages of the disease. The nucleus of treatment is patient education, physical activity and therapeutic exercise, together with weight control in overweight or obese patients. Self-care by the individual and by the family is fundamental in day-to-day patient management. The use of physical therapies, technical aids (walking sticks, etc.) and simple analgesics, opium alkaloids, and antiinflammatory drugs have demonstrated effectiveness in controlling pain, improving physical function and quality of life and their use is clearly indicated in the treatment of osteoarthritis. Conservative surgery and joint replacement is indicated when treatment goals are not achieved in specific patients. PMID:24467960

  16. Patient comfort and staff facilitation.

    PubMed

    Driver, J; Pennell, L

    1983-01-01

    What can be done to help staff work with patients in such a way that patients' psychological functioning can be maintained at a high level? What suggestions can be given to help staff members support the patient's self-esteem and thus enhance the self-image? Those who work directly with patients in a nursing home have chosen this work because they care about persons and about their comfort. These motivations plus continued awareness of the patient's perceptions, feelings, and attitudes will enable more patients to be psychologically supported while they live in a health care facility. It is hoped that as we go about seeking to facilitate the patient's return to health, we might also be aware of the needs of the staff, for only as they have their own needs fulfilled can they serve another. PMID:10283902

  17. [Psychotropic medication of elderly patients].

    PubMed

    Kumagai, Ryo; Iseki, Eizo

    2012-01-01

    Drugs medicated for elderly patients show different pharmacokinetics from young patients, because of change in the makeup of bodies, decrease of albumin, increase of alpha-1-acid glycoprotein, hypofunction of metabolism, and excretion by aging. In addition, complications or other concomitant drugs influence their pharmacokinetics. Therefore, when we take medication for elderly patients, we need consideration different from young patients. Psychiatric symptoms which frequently occurred in elderly patients are dementia (including behavioral and psychological symptoms of dementia), depressive state, sleep disturbance, and delirium. As warned by Food and Drug Administration, some studies reported that using antipsychotics for elderly patients with dementia increase their death rate. We have to give informed consent sufficiently to patients and their families. PMID:22413509

  18. Effects of Patient Navigation on Patient Satisfaction Outcomes.

    PubMed

    Post, Douglas M; McAlearney, Ann Scheck; Young, Gregory S; Krok-Schoen, Jessica L; Plascak, Jesse J; Paskett, Electra D

    2015-12-01

    Patient navigation (PN) may reduce cancer health disparities. Few studies have investigated the effects of PN on patient-reported satisfaction with care or assessed patients' satisfaction with navigators. The objectives of this study are to test the effects of PN on patient satisfaction with cancer care, assess patients' satisfaction with navigators, and examine the impact of barriers to care on satisfaction for persons with abnormal cancer-related screening tests or symptoms. Study participants included women and men with abnormal breast, cervical, or colorectal cancer screening tests and/or symptoms receiving care at 18 clinics. Navigated (n=416) and non-navigated (n=292) patients completed baseline and end-of-study measures. There was no significant difference between navigated and non-navigated patients in change in patient satisfaction with cancer care from baseline to exit. African-American (p<0.001), single (p=0.03), low income (p<0.01), and uninsured patients (p<0.001) were significantly less likely to report high patient satisfaction at baseline. A significant effect was found for change in satisfaction over time by employment status (p=0.04), with full-time employment showing the most improvement. The interaction between satisfaction with navigators and satisfaction with care over time was marginally significant (p=0.08). Baseline satisfaction was lower for patients who reported a barrier to care (p=0.02). Patients reporting other-focused barriers (p=0.03), including transportation (p=0.02), had significantly lower increases in satisfaction over time. Overall, results suggested that assessing barriers to cancer care and tailoring navigation to barrier type could enhance patients' experiences with health care. PN may have positive effects for healthcare organizations struggling to enhance quality of care. PMID:25510369

  19. Thromboprophylaxis with dalteparin in medical patients: which patients benefit?

    PubMed

    Cohen, Alexander T; Turpie, Alexander G G; Leizorovicz, Alain; Olsson, Carl-Gustav; Vaitkus, Paul T; Goldhaber, Samuel Z

    2007-05-01

    It is unclear whether thromboprophylaxis produces a consistent risk reduction in different subgroups of medical patients at risk from venous thromboembolism. We performed a retrospective, post hoc analysis of 3706 patients enrolled in the PREVENT study. Patients were at least 40 years old with an acute medical condition requiring hospitalization for at least 4 days and had no more than 3 days of immobilization prior to enrolment. Patients received either subcutaneous dalteparin (5000 IU) or placebo once daily. The primary end point was the composite of symptomatic deep vein thrombosis (DVT), pulmonary embolism, asymptomatic proximal DVT, or sudden death. Primary diagnosis subgroups were acute congestive heart failure, acute respiratory failure, infectious disease, rheumatological disorders, or inflammatory bowel disease. All patients, except those with congestive heart or respiratory failure, had at least one additional risk factor for venous thromboembolism. A risk reduction was shown in patients receiving dalteparin versus placebo. The relative risk (RR) was 0.73 in patients with congestive heart failure, 0.72 for respiratory failure, 0.46 for infectious disease, and 0.97 for rheumatological disorders. The RR was 0.52 in patients aged > or = 75 years, 0.64 in obese patients, 0.34 for patients with varicose veins, and 0.71 in patients with chronic heart failure. No subgroup had a significantly different response from any other. Importantly, multivariate analysis showed that all patient groups benefited from thromboprophylaxis with dalteparin. Our findings, therefore, support the broad application of thromboprophylaxis in acutely ill hospitalized medical patients. PMID:17615800

  20. Medications in older patients.

    PubMed Central

    Hobson, M

    1992-01-01

    Adverse drug reactions are common in persons aged 65 and older and are associated with increased morbidity and mortality. A heightened susceptibility to adverse reactions is due to a number of factors, including an increased incidence of disease, multiple drug use, and altered pharmacokinetic and pharmacodynamic properties of many drugs. The risk of drug interactions increases with the number of medications taken. Adverse drug reactions can be prevented through prudent prescribing practices, patient education, and adequate monitoring of drug efficacy and side effects. Several types of medications are of particular concern, including many antihypertensive agents, drugs with anticholinergic effects, psychoactive medications, and nonsteroidal anti-inflammatory drugs. Some drugs, such as histamine H2-receptor antagonists, are relatively safe but are overprescribed. Data regarding the risks associated with these problem drugs are presented, with recommendations for safe and effective treatment alternatives. PMID:1309183

  1. [Nutrition for diabetic patients].

    PubMed

    Schindler, Karin; Brix, Johanna; Dämon, Sabine; Hoppichler, Friedrich; Kruschitz, Renate; Toplak, Hermann; Ludvik, Bernhard

    2016-04-01

    Evidence demonstrates that medical diabetes treatment has to be accompanied by lifestyle modifications. Structured nutrition interventions and increased physical activity will help patients to normalise, respectively maintain their body weight. The main target of a diabetes therapy is aimed at achieving normal or nearly normal blood glucose levels. Reaching this goal may be facilitated by the following nutritional patterns: Using mainly carbohydrates from vegetables, whole grains, legumes and fruits, Restriction of mono- and disaccharides are often important factors in normalising body weight and blood glucose, Reduction of dietary fat could be indicated. However, the primary goal is the limitation of saturated fatty acids which to high percentage are consumed with animal products. There is not sufficient evidence to recommend a dietary protein consumption of more than 20 % of energy intake. Individuals with diabetes should be aware of the importance of acquiring daily vitamin and mineral requirements. Natural food sources should be preferred. PMID:27052240

  2. Prescription Opioid Analgesics: Promoting Patient Safety with Better Patient Education.

    PubMed

    Costello, Margaret

    2015-11-01

    Patients expect and deserve adequate postoperative pain relief. Opioid analgesics are widely used and effective in controlling postoperative pain, but their use poses risks that many patients don't understand and that all too often result in adverse outcomes. Inappropriate and often dangerous use of prescription medication has increased sharply in the past two decades in the United States. Patients and caregivers must have an adequate understanding of safe use, storage, and disposal of opioids to prevent adverse drug events in patients and others. Nurses play a key role in providing this patient education. This article provides a case study that highlights the risks and important aspects of opioid medication use in the postoperative patient. PMID:26510070

  3. Patient Navigators’ Reflections on the Navigator-Patient Relationship

    PubMed Central

    Phillips, Sara; Nonzee, Narissa; Tom, Laura; Murphy, Kara; Hajjar, Nadia; Bularzik, Charito; Dong, Xinqi

    2014-01-01

    Patient navigation emerged as a strategy to reduce cancer disparities among low-income and minority patients and has demonstrated efficacy in improving clinical outcomes. Observational studies have contributed valuable evaluations of navigation processes and tasks; however, few have offered in-depth reflections about the relationship between patient and navigator from the navigators’ perspective. These approaches have addressed the emotional and relational components of patient navigation through the lens of process factors, relegating the navigator-patient relationship to a siloed, compartmentalized functionality. To expand upon existing task-oriented definitions of navigation, we conducted qualitative interviews among community-based patient navigators who coordinated care for uninsured, predominantly Hispanic, women receiving cancer screening and follow-up care in a county outside Chicago. Interviews were recorded, transcribed, and analyzed for themes within the navigator-patient relationship domain. The main themes that emerged centered on relational roles, relational boundaries, and ideal navigator relational qualities. While patient navigators described engaging with patients in a manner similar to a friend, they stressed the importance of maintaining professional boundaries. Navigators’ support assisted patients in bridging their hospital and community lives, a result of navigators’ investment in both hemispheres. We conclude that the navigator-patient relationship is not a self-contained utility, but rather the medium through which all other navigator functions are enabled. These insights further characterize the navigator-patient relationship, which will help shape the development of future navigation programs and support the need for further research on the impact of relationship factors on clinical and psychosocial outcome measures. PMID:24493636

  4. [Autonomy of the patient with chronic diseases: from passive patient to active patient].

    PubMed

    González Mestre, Assumpció

    2014-01-01

    Due to social, economic and cultural changes, there has been a transformation of Health Services around the world. A new figure has emerged from this: the Active Patient, more responsible, with more information and willing to change his life as a chronic patient. In order to respond to this new situation, several countries have established initiatives such as self-reliance programmes for chronic patients. The aim of this article is to underline the Expert Patient Programme Catalonia(®) and to explain its operation and the results obtained up until now. The purpose of this program is to improve the experience of chronic disease by patients, from meetings in which an expert patient provides his knowledge and experiences to a group of patients with the same disease, with the aim of promoting changes in habits and lifestyles that improve the quality of life and the coexistence of the person with his chronic process. PMID:24433841

  5. Patients' views of nurses' competence.

    PubMed

    Calman, Lynn

    2006-12-01

    This study examines, from the patients' perspective, what is meant by competent nursing and how, with this perspective in mind, patients would view the prospect of assessing the competence of nurses. There is a little empirical research that clarifies professional competence from the patient's perspective. Nursing curricula in the UK have shifted attention to 'competencies' as the outcome of nurse education and, in an era of patient involvement, their views are important to investigate. The study utilises a grounded theory approach. Data were collected in Central Scotland between 2001 and 2003. Twenty-seven patients participated. Data were analysed, in keeping with the grounded theory tradition, utilising the constant comparative method. Patients described the foundation of competent nursing practice as technical care and nursing knowledge. Patients assume that technical care is competent as safe guards are considered to be in place to protect patients. When technical competence is assumed, interpersonal attributes become the most important indicator of the quality of nursing care. The results of this study highlight uncertainty about whether patients feel able to assess the competence of nurses. The results of this study may have implications for nurses internationally when trying to involve patients in the assessment of nurses. PMID:17014931

  6. [Vascular access in diabetic patients. Are these patients "difficult"?].

    PubMed

    Gołębiowski, Tomasz; Weyde, Wacław; Kusztal, Mariusz; Porażko, Tomasz; Augustyniak-Bartosik, Hanna; Madziarska, Katarzyna; Krajewska, Magdalena; Koniński, Przemysław; Sydor, Antoni; Letachowicz, Krzysztof; Klinger, Marian

    2015-01-01

    Diabetics with stage V chronic kidney disease (CKD) on hemodialysis (HD) are considered as "difficult patients", because of problems with creation of the vascular access. There is controversy regarding the results and recommendations for preparation of the vascular access in these patients. The aim of this retrospective study was to evaluate the results of creating different types of arteriovenous fistula (AVFs) in consecutive series of patients starting dialysis treatment. The analysis was performed in 741 patients (385 females and 356 males), average age 61.4±7 years, who started dialysis treatment in our department between January 2005 and December 2012. Native AVFs were created in all patients. No patients received an AVF requiring synthetic graft material. The number of patients with diabetic nephropathy was 166 (22.4%). Among them, 30 (18%) had type 1 diabetes and 136 (82%) type 2. In this group the occurrence of calcification in the forearm artery was estimated on the basis of physical examination, Allan's test, Doppler ultrasound and forearm X-ray. In a subgroup of patients with atherosclerotic changes in the arterial system the frequency of failed AVFs was analyzed. These results were compared with the group without diabetes. The number of procedures necessary for successfu AVF creation and type of access was counted in both groups. The assessment of the procedure frequency and AVF location in diabetic and in non-diabetic patients was made by χ² test with Yates correction. In the group of 166 patients with diabetes, in 100 cases (60%) atherosclerotic changes in forearm arteries were observed. In a subgroup of 30 patients with type 1 diabetes atherosclerosis was observed in 17 adults (57%). In this subgroup creation of a suitable forearm AVF in the first procedure in 9 patients was possible and in the other 8 cases the atherosclerotic changes necessitated repeated procedures and were an important obstacle to create the AVF. In the subgroup of 136 patients with type 2 diabetes, atherosclerosis in the forearm artery was observed in 83 cases (61%). In this subgroup the creation of a native AVF was possible in 40 patients during the first procedure and in 43 cases additional intervention was needed, but only in 8 cases was atherosclerosis the cause of fistula failure. Generally, among 166 patients only in 16 cases (10%) did atherosclerosis present an important obstacle for AVF creation, but the number of necessary procedures to create one functioning AVF was significantly greater in this population (2 v 2.7, p=0.0001). A functioning AVF in patients with diabetes was found significantly frequently in the arm localization in comparison to non-diabetic patients (4.3 v 2%, p=0.0478). Atherosclerotic changes in forearm arteries in diabetic patients appear in 60% of hemodialysis patients and make it difficult to create an AVF only in 10% of diabetic patients, but it is possible to create a native AVF in 90% of diabetic patients, although this requires more procedures. In patients with diabetes, AVF in the wrist region should be preferred. PMID:26270518

  7. Six hundred patients with gallstones.

    PubMed Central

    Warwick, D. J.; Thompson, M. H.

    1992-01-01

    A total of 610 patients with gallstones were treated over an 8-year period in a single surgical unit. Of these patients, 384 had cholecystectomy, 86 surgical duct exploration and 140 endoscopic sphincterotomy. Four patients died after cholecystectomy (1%), but there were no deaths after the treatment of duct stones. The proportion of patients with duct stones having endoscopic sphincterotomy with the gallbladder in situ rose considerably during the 8-year period, from about 20% to 75%. Gallstones tend to present in the elderly as duct stones, and in the young as gallbladder stones. The majority of patients over 75 years of age had endoscopic sphincterotomy, whereas the younger patients were usually treated surgically. PMID:1616266

  8. Lipoplasty in the bulimic patient.

    PubMed

    Willard, S G; McDermott, B E; Woodhouse, L M

    1996-08-01

    Recent cases in our Eating Disorders Clinic suggest that patients diagnosed with bulimia nervosa seeking surgical fat removal may be exhibiting a variant of the purging behavior seen in bulimic patients. These same patients exhibit historical or concurrent abuse of laxatives and/or diuretics or self-induced vomiting in a pathologic attempt to obtain or maintain an idealized body image. This paper presents two case studies that illustrate the bulimic patient's compulsive quest for lipectomy with unrealistic expectations that surgical alteration of the body will be an emotional and physical panacea. Plastic surgeons must be cautioned regarding this potential manifestation of bulimia nervosa and the dangers inherent in colluding with the patient in a pathologic request for surgery. It is important for plastic surgeons to recognize appropriate use of lipectomy as an alternative to traditional purging behavior in the bulimic patient. PMID:8764715

  9. [Interpersonal patterns in obese patients].

    PubMed

    Kiesewetter, Sybille; Köpsel, Andrea; Pfeiffer, Andreas F; Köpp, Werner; Kastner, Sabrina; Orth-Gomér, Kristina; Deter, Hans-Christian

    2014-07-01

    In the last decades the number of obese and pre-obese patients in Western industrial nations increased. Obese patients have been largely unsuccessful in losing weight over time, but the causes of their difficulties and the causes of their obesity have remained unclear. We examined whether the attachment style and its interaction with interpersonal processes would shed light on this question. We analyzed 107 obese or pre-obese patients before the start of a weight loss intervention program. We used the Adult Attachement Prototype Rating (AAPR)-interview and related questionnaires (Helping Alliance Questionnaire (HAQ), Inventory of Interpersonal Problems (IIP-D), Brief Symptom Inventory (BSI)). According to the AAPR-rating 54% of the patients were secure and 46% insecure attached. The results suggest that insecure attachment style has an impact on psychic strain in obese patients unrelated to weight, gender and age. Additionally the insecure attachment style influences the therapeutic alliance experienced by patients and the therapist. PMID:24446185

  10. Mortality among patients with schizophrenia.

    PubMed

    Simpson, J C; Tsuang, M T

    1996-01-01

    Results from a 40-year followup study of psychotic patients are presented to illustrate some of the major findings and unanswered questions about excess mortality in schizophrenia and to suggest analytical approaches that take full account of the potential effects of sample heterogeneity. In this study, hospital-diagnosed schizophrenia patients were at increased mortality risk whether or not they met research criteria for schizophrenia. In addition, mortality outcomes of several major diagnostic groups were similar despite substantial clinical and demographic differences between the groups at baseline. These results suggest that both diagnosis-specific and nondiagnostic factors are needed to account for excess mortality in patients with major psychiatric disorders. The issue of heterogeneity is also crucial for the clinical purposes of predicting and ultimately reducing the mortality risk of psychiatric patients, for example, in delineating profiles of high-risk patients who are not necessarily typical of other patients with the same diagnosis. PMID:8873299

  11. Trading patients. Lessons from Scandinavia.

    PubMed

    Botten, Grete; Grepperud, Sverre; Nerland, Sölve Mikal

    2004-09-01

    The next few decades will bring about more trade in services, among them health care. This paper describes a recent project on cross-border trading of patients initiated by the Norwegian parliament (The Patient Bridge). This health policy reform met some resistance among hospital physicians. However, patients were willing to participate if being properly informed and supported by local health care workers. The Patient Bridge turned out to be a relatively expensive project partly because of the transaction costs involved (transportation and escort) and partly because of high treatment costs. Excessive treatment costs were a result of insufficient cost-consciousness in the purchasing organization. The Patient Bridge revealed large price differentials not only between Norwegian and foreign hospitals, but also between hospitals abroad, even within the same country. This finding points to the possibilities of reaching mutual gains from trading patients across borders. PMID:15276311

  12. Patient discharge referral: interdisciplinary collaboration.

    PubMed Central

    Prophet, C. M.

    1992-01-01

    The INFORMM (Information Network For Online Retrieval & Medical Management) patient discharge referral form is interdisciplinary in scope. The initial automated form, implemented on 41 general inpatient care units as of December, 1991, involved the collaboration of the departments of Nursing, Social Services, Medical Records, and Patient Registration. As development proceeds, it is expected that other clinical disciplines will contribute additional data and information to augment and complement the content of the patient discharge referral form. PMID:1482892

  13. Thromboprophylaxis in immobilized medical patients.

    PubMed

    Vaitkus, Paul T

    2004-03-30

    Venous thromboembolism accounts for a large number of preventable deaths. The majority of these events occur in medical patients, but medical thromboprophylaxis remains underutilised in this population. The purpose of this review is to examine the results of recent clinical trials of low molecular weight heparins in the prevention of venous thromboembolic disease in medical patients. The available data make a compelling case in favor of widespread use of low molecular weight heparin in medical patients. PMID:15096323

  14. Meeting deaf patients' communication needs.

    PubMed

    Dickson, Megan; Magowan, Ruth; Magowan, Ruth

    Effective communication between nurses and patients is a vital part of safe and effective nursing care. However, few health professionals receive training in how to communicate with Deaf people; as a result, attempts to communicate with Deaf patients is often inappropriate and undertaken without knowledge or understanding of their communication needs. This article examines the literature on ways in which Deaf patients experience communicating with, and receive care from, nurses. PMID:26016132

  15. Sinusitis: Special Considerations for Aging Patients

    MedlinePlus

    ... ENT Doctor Near You Sinusitis: Special Considerations for Aging Patients Sinusitis: Special Considerations for Aging Patients Patient ... for evaluation and possible surgical management. Sources For Aging Patients: Administration on Aging (AoA), U.S. Department of ...

  16. Thyroid Disease in the Older Patient

    MedlinePlus

    ... these patients, without treatment unless they are symptomatic. HYPOTHYROIDISM IN THE OLDER PATIENT Hypothyroidism is very common ... is given. TREATMENT OF THE OLDER PATIENT WITH HYPOTHYROIDISM As with the younger patient, pure synthetic thyroxine ( ...

  17. [Laparoscopic cholecystectomy in older patients].

    PubMed

    Modrzejewski, A; Borowski, M

    1993-11-01

    Operational risk of laparoscopic cholecystectomy in elderly patients has been retrospectively evaluated in 600 consecutive patients. Statistical correlation between age and risk factors as: biliary duct disease, adhesions, perforation of the gall bladder, necessity of extending of the incision, duration of the procedure, body temperature after LCh, morbidity, conversion rate from LCh to open cholecystectomy, has been tested. Despite of greater technical difficulties in elderly patients morbidity was not increased significantly. LCh in aged patients with cholecystitis is a safe method of choice. PMID:7817569

  18. Erectile dysfunction in hemodialysis patients.

    PubMed

    Gorsane, Imen; Amri, Nadia; Younsi, Fathi; Helal, Imed; Kheder, Adel

    2016-01-01

    Erectile dysfunction (ED) is a common problem seen among patients on hemodialysis (HD), but it is still a taboo subject in our country. The attention given to this sexual problem remained low, and the prevalence of ED among these patients has not been well characterized. We carried out this study in order to determine the prevalence and severity of ED in HD patients. We conducted a descriptive cross-sectional study in our HD unit in March 2013. ED was evaluated using the International Index Erection Function. Thirty patients with a mean age of 49.1 years were eligible for this study. The main causes of chronic kidney disease were hypertension (62.5%) and diabetes (41.6%). The prevalence of ED was 80%, including 33.3% severe ED. Plasma levels of gonadotropins: luteinizing hormone (LH), follicule-stimulating hormone were in the standards except for one patient who had an elevated level of LH. Prolactin was elevated in four cases. ED was present in 8.4% of patients before the discovery of renal failure and in 91.6% of patients at the beginning of dialysis. For 19 patients (79.1%), the ED had increased during the dialysis sessions. A significant number of our HD patients presented with ED of varying degrees. Nephrologists should pay attention to the problem of ED in order to improve the quality of their life. PMID:26787562

  19. Pharmacists see patients through discharge.

    PubMed

    2014-02-01

    At The Nebraska Medical Center in Omaha, pharmacists are part of a multidisciplinary team and see many patients in person starting on Day 1. Every patient history is either taken by a pharmacist or reviewed and approved by the pharmacists. They review the discharge prescriptions, conduct medication reconciliation, and educate the patients on their medications and the importance of taking them as directed. Case managers work with pharmacists to identify patients who are at high risk for readmissions and need follow-up calls and collaborated to develop a medication instruction sheet. PMID:24505833

  20. Elderly patients with pancreatic cancer.

    PubMed

    Kougioumtzopoulou, Andromachi S; Syrigos, Kostas N; Saif, Muhammad Wasif

    2014-07-01

    Pancreatic cancer marked significant increase of incidence during the last decades in the elderly population. Despite the certain increase of incidence there are no international guidelines for elderly patients who are suffering from pancreatic cancer. During the ASCO Annual Meeting 2014, two abstracts focusing on elderly patients suffering from different histological types of pancreatic cancer were presented. The first retrospective study (Abstract #4119) showed the benefit of the systemic treatment on overall survival for elderly patients with stage IV pancreatic adenocarcinoma. The second retrospective study (Abstract #4112) demonstrates the positive effect of somatostatin analogue (octreotide-LAR) treatment on overall survival for elderly patients with neuroendocrine pancreatic carcinoma. PMID:25076333

  1. Medical liability and patient safety.

    PubMed

    Sage, William M

    2003-01-01

    Political debate over medical malpractice reform seldom takes meaningful account of its policy context, including the emerging science of patient safety. Instead, stakeholders on both sides use the rhetoric of patient safety to support entrenched positions on hardened proposals such as capping damages and limiting access to information about errors. Despite its déjà vu quality, the current malpractice crisis can only be understood and addressed as the product of changes in the health care system since the last crisis nearly twenty years ago--changes that also informed the patient safety movement. Patient safety may therefore serve as a bridge between medical liability and health policy. PMID:12889746

  2. Nutritional assessment of surgical patients.

    PubMed

    Brown, C S; Stegman, M R

    1988-10-01

    In order to test the sensitivity and specificity of the East Orange Nutritional Screening Form (EONSF), nutritional assessments were performed on a random sample of 10% of general medical/surgical admissions at a large midwestern veteran's administration hospital. Patients were followed until discharge to determine if they met the standard criteria of additional nutritional support. The tool correctly identified patients at nutritional risk (sensitivity) 95% of the time and patients not at nutritional risk (specificity) 89% of the time. It proved to be an effective, low-cost tool for identifying patients at risk and for planning appropriate nutritional strategies. PMID:3146037

  3. Cultural competence: serving Latino patients.

    PubMed

    Poon, Amy W; Gray, Katherine V; Franco, Gina C; Cerruti, Domenica M; Schreck, Michael A; Delgado, Eliana D

    2003-01-01

    Culturally competent strategies are necessary as populations in many countries become increasingly diverse. In the United States, Latinos are the fastest-growing minority group. In this study, the authors interviewed Latino patients and families, collected demographics from 570 patients in pediatric orthopedic practices in California, and conducted population census and literature reviews. Based on these sources of information, the authors identified barriers to health care for Latino pediatric orthopedic patients and propose culturally competent strategies to overcome these barriers. This approach can be applied to other populations so that culturally competent care is available to patients from all ethnic, racial, and cultural backgrounds. PMID:12826958

  4. DENGUE INFECTION IN ELDERLY PATIENTS.

    PubMed

    Tiawilai, Thawat; Tiawilai, Anongrat; Nunthanid, Somboon

    2015-01-01

    From 2005 to 2013, there were 15 dengue patients aged over 60 years old who were admitted to Photharam Hospital, Ratchaburi, Thailand. Ten were females and five were males. Nine had dengue fever (DF), and 6 had dengue hemorrhagic fever (DHF). A trending shift in age group towards adults has been seen during the past decades. No deaths were seen in these elderly patients with dengue disease, indicating early recognition and effective management of these dengue patients. The trend towards higher age in dengue patients is a problem of concern, which needs further elaboration. PMID:26506744

  5. Effective writing that attracts patients.

    PubMed

    Baum, Neil

    2015-01-01

    Doctors today not only must communicate verbally, they must also realize that the written word is important to their ability to connect with the patients that they already have and also to attract new patients. Doctors will be expected to write blogs, to create content for their Web sites, to write articles for local publications, and even to learn to express themselves in 140 characters or less (i.e., Twitter). This article presents 10 rules for selecting the right words to enhance your communication with existing patients and potentially to attract new patients to your practice. PMID:26062324

  6. [Nutritional assessment for hospitalized patients].

    PubMed

    Henríquez Martínez, T; Armero Fuster, M

    1991-01-01

    A review of the following points was performed: Factors favouring the development and presence of malnutrition among hospitalized patients. Useful parameters in nutritional evaluation. Types of malnutrition. The Chang nutritional evaluation protocol is used in our Hospital, which is simple, inexpensive, reliable, specific and easily reproduced. This is based on five variables (three anthropometric and two biochemical), randomized and based on reference tables and values. A study was made on data corresponding to 70 patients, in whom a prevalence of malnutrition was observed in critical patients. The patients were classified based on three different definitive possibilities (Marasmo, Kwashiorkor and combined), and three grades of malnutrition (slight, moderate and severe). PMID:2070010

  7. Patient Transport via Commercial Airlines

    PubMed Central

    Macnab, Andrew John

    1992-01-01

    Because the frequency of patient transport from one hospital to another is increasing and the popularity of air travel continues to rise, physicians should be aware of the procedures for patient transport by commercial airlines. Major airlines in Canada have experienced personnel and established procedures that facilitate the transportation of patients with special medical needs. By working with the airline medical health officers and using up-to-date equipment, physicians can achieve safe, cost-effective transport of appropriate patients via commercial aircraft. PMID:21221401

  8. Patient or customer?

    PubMed

    Parker, J M

    1999-01-01

    This paper investigates caring in practice within the context of the global imperative of increasing rationalisation of care based on an economic ethic. The notion of the global marketplace has spread to the domain of health services, so that 'health' has come to be seen as a commodity, with the body as its site, and the 'patient' a customer; clinicians work to construct standard pathways through the healthcare supermarket. The challenge for nurses is to work within but also to challenge and resist the reductionist impetus of economically based and commercially driven approaches to health care. They must retain the sense of the value of the wholeness of the person, the deeply personal and profoundly significant professional-recipient relationship, and find ways of demonstrating their capacity to deliver high-quality care in a cost-effective way. Proper and appropriate accountability is a key strategy to maintaining quality nursing as a significant aspect of care. The expansion of the role of the advanced practice nurse is very useful in providing holistic and cost-effective care, though there are currently limitations to scope of practice that need to be removed. The metaphor of the marketplace, underpinned by powerful global economic forces, can draw us into unthinking compliance with its imperatives--but other metaphors are available. Metaphor and creativity are linked, and we need to consider how the creative use of language can facilitate the emergence of new ways of understanding in health care. PMID:10401282

  9. Unconventional patient moves.

    PubMed

    O'Neill, E J

    2001-05-01

    EMS is a dynamic occupation. Every day, we face new challenges. Without some "outside-the-box" thinking, it is difficult, if not impossible, to keep up. In this article, we have discussed only a few of the "tools" we apply in adverse situations. As members of the only hospital-based EMS heavy-rescue unit in the United States, we believe collectively that our primary mission is safety, first for providers and then for patients. Much of what is carried on an average heavy-rescue unit goes unused or is used only rarely. If we can take tools, equipment and techniques that we would ordinarily only apply for their most obvious use and use them in a unique and unconventional way while promoting the safety of all parties involved, then we have accomplished our primary objective. Don't let your equipment collect dust because you are only willing to use it for obvious reasons. Apply it to every rescue situation you safely can. PMID:11373916

  10. Patient-Centered Communication and Prognosis Discussions with Cancer Patients

    PubMed Central

    Shields, Cleveland G.; Coker, Casey J.; Poulsen, Shruti S.; Doyle, Jennifer M.; Fiscella, Kevin; Epstein, Ronald M; Griggs, Jennifer J.

    2009-01-01

    Objective To examine physician communication associated with prognosis discussion with cancer patients. Methods We conducted a study of physician-patient communication using trained actors. Thirty-nine physicians, including 19 oncologists and 20 family physicians participated in the study. Actors carried two hidden digital recorders to unannounced visits. We coded recordings for eliciting and validating patient concerns, attentive voice tone, and prognosis talk. Results Actor adherence to role averaged 92% and the suspected detection rate was 14%. In a multiple regression, eliciting and validating patient concerns (β=.40, C.I. = 0.11-0.68) attentiveness (β=.32, C.I. = 0.06-0.58) and being an oncologist vs. a family physician (β=.33, C.I. = 0.33 - 1.36) accounted for 46% of the variance in prognosis communication. Conclusion Eliciting and validating patient concerns and attentiveness voice tone is associated with increased discussion of cancer patient prognosis as is physician specialty. Practice Implications Eliciting and validating patient concerns and attentive voice tone may be markers of physician willingness to discuss emotionally difficult topics. Educating physicians about mindful practice may increase their ability to collect important information and to attend to patient concerns. PMID:19819098

  11. Patient Education Leads to Better Care for Heart Patients.

    ERIC Educational Resources Information Center

    Rosenberg, Stanley G.

    The staff of a heart and circulatory disease program of a State department of health conducted a special project at a city hospital which showed that a well-organized treatment and education program for patients with congestive heart failure increased the patient's knowledge of his disease, medication, and diet as well as his adherence to a…

  12. What Do Patients Want? Patient Preference in Wound Care

    PubMed Central

    Corbett, Lisa Q.; Ennis, William J.

    2014-01-01

    Patient preferences are statements made or actions taken by consumers that reflect their desirability of a range of health options. The concept occupies an increasingly prominent place at the center of healthcare reform, and is connected to all aspects of healthcare, including discovery, research, delivery, outcome, and payment. Patient preference research has focused on shared decisions, decisional aids, and clinical practice guideline development, with limited study in acute and chronic wound care populations. The wound care community has focused primarily on patient focused symptoms and quality of life measurement. With increasing recognition of wound care as a medical specialty and as a public health concern that consumes extensive resources, attention to the preferences of end-users with wounds is necessary. This article will provide an overview of related patient-centered concepts and begin to establish a framework for consideration of patient preference in wound care. PMID:25126474

  13. Patients as partners, patients as problem-solvers.

    PubMed

    Young, Amanda; Flower, Linda

    2002-01-01

    This article reports our ongoing work in developing a model of health care communication called collaborative interpretation, which we define as a rhetorical practice that generates building blocks for a more complete and coherent diagnostic story and for a collaborative treatment plan. It does this by situating patients as problem-solvers. Our study begins with an analysis of provider-patient interactions in a specific setting-the emergency department (ED) of an urban trauma-level hospital- where we observed patients and providers miscommunicating in at least 3 distinct areas: over the meaning of key terms, in the framing of the immediate problem, and over the perceived role of the ED in serving the individual and the community. From our observations, we argue that all of these miscommunications and missed opportunities are rooted in mismatched expectations on the part of both provider and patient and the lack of explicit comparison and negotiation of expectations-in other words, a failure to see the patient-provider interaction as a rhetorical, knowledge-building event. In the process of observing interactions, conversing with patients and providers, and working with a team of providers and patients, we have developed an operational model of communication that could narrow the gap between the lay public and the medical profession-a gap that is especially critical in intercultural settings like the one we have studied. This model of collaborative interpretation (CI) provides strategies to help patients to represent their medical problems in the context of their life experiences and to share the logic behind their health care decisions. In addition, CI helps both patient and provider identify their goals and expectations in treatment, the obstacles that each party perceives, and the available options. It is adaptableto various settings, including short, structured conversations in the emergency room, extended dialogue between a health educator and a patient in a clinical setting, and group discussions in support groups, community groups, or health education classrooms. PMID:11853210

  14. Patient Perspectives on Spirituality and the Patient-physician Relationship

    PubMed Central

    Hebert, Randy S; Jenckes, Mollie W; Ford, Daniel E; O'Connor, Debra R; Cooper, Lisa A

    2001-01-01

    OBJECTIVE To identify the preferences and concerns of seriously ill patients about discussing religious and spiritual beliefs with physicians. DESIGN Three focus group discussions with patients who had experienced a recent life-threatening illness. Discussions were audiotaped, transcribed verbatim, and reviewed independently by two investigators to identify discrete comments for grouping into domains. A third investigator adjudicated differences in opinion. Comments were then independently reviewed for relevance and consistency by a health services researcher and a pastoral counselor. SETTING Academic medical center. PARTICIPANTS Referred sample of 22 patients hospitalized with a recent life-threatening illness. MEASUREMENTS AND MAIN RESULTS Almost all of the 562 comments could be grouped into one of five broad domains: 1) religiosity/spirituality, 2) prayer, 3) patient-physician relationship, 4) religious/spiritual conversations, and 5) recommendations to physicians. God, prayer, and spiritual beliefs were often mentioned as sources of comfort, support, and healing. All participants stressed the importance of physician empathy. Willingness to participate in spiritual discussions with doctors was closely tied to the patient-physician relationship. Although divided on the proper context, patients agreed that physicians must have strong interpersonal skills for discussions to be fruitful. Physician-initiated conversation without a strong patient-physician relationship was viewed as inappropriate and as implying a poor prognosis. CONCLUSION Religion and spirituality are a source of comfort for many patients. Although not necessarily expecting physicians to discuss spirituality, patients want physicians to ask about coping and support mechanisms. This exploratory study suggests that if patients then disclose the importance of spiritual beliefs in their lives, they would like physicians to respect these values. PMID:11679036

  15. National survey of hospital patients.

    PubMed Central

    Bruster, S.; Jarman, B.; Bosanquet, N.; Weston, D.; Erens, R.; Delbanco, T. L.

    1994-01-01

    OBJECTIVE--To survey patients' opinions of their experiences in hospital in order to produce data that can help managers and doctors to identify and solve problems. DESIGN--Random sample of 36 NHS hospitals, stratified by size of hospital (number of beds), area (north, midlands, south east, south west), and type of hospital (teaching or non-teaching, trust or directly managed). From each hospital a random sample of, on average, 143 patients was interviewed at home or the place of discharge two to four weeks after discharge by means of a structured questionnaire about their treatment in hospital. SUBJECTS--5150 randomly chosen NHS patients recently discharged from acute hospitals in England. Subjects had been patients on medical and surgical wards apart from paediatric, maternity, psychiatric, and geriatric wards. MAIN OUTCOME MEASURES--Patients' responses to direct questions about preadmission procedures, admission, communication with staff, physical care, tests and operations, help from staff, pain management, and discharge planning. Patients' responses to general questions about their degree of satisfaction in hospitals. RESULTS--Problems were reported by patients, particularly with regard to communication with staff (56% (2824/5020) had not been given written or printed information); pain management (33% (1042/3162) of those suffering pain were in pain all or most of the time); and discharge planning (70% (3599/5124) had not been told about warning signs and 62% (3177/5119) had not been told when to resume normal activities). Hospitals failed to reach the standards of the Patient's Charter--for example, in explaining the treatment proposed and giving patients the option of not taking part in student training. Answers to questions about patient satisfaction were, however, highly positive but of little use to managers. CONCLUSIONS--This survey has highlighted several problems with treatment in NHS hospitals. Asking patients direct questions about what happened rather than how satisfied they were with treatment can elucidate the problems that exist and so enable them to be solved. PMID:7819893

  16. [The physician, patient and antibiotics].

    PubMed

    Pechre, Jean-Claude

    2004-01-01

    More than 3.000 randomized patients, who received an antibiotic course for a mild respiratory infection in the last 2 months have been interviewed in 4 European countries about their perceptions of antibiotic therapy and the doctor's skills. Six attitudinal dimensions related to the doctor identified 4 patients type: Involved (30 %), Deferents (23%), Ignored (13%) and Critical (17%). Involved and Deferent patients knew better the rules of good antibiotic use (p<0,01), were more compliant (p<0,01), and received more accurater information from the doctor (p<0,01). Ignored patients keep left over antibiotics for uncontrolled further use most often (p<0,01). A large majority of patients, whatever the category, believed that a flu should be treated with an antibiotic. Germany includes more involved patients, the highest rate of confidence in physician's skills, who was the most informative, but they also had less people knowing the uselessness of antibiotics in flu. Spaniards had more propensity to expect antibiotics from their doctor, showed the lesser level of confidence in their physician's skill, and were the most prone to claim for the benign character of their infection. Critical patients were mostly recruited in France and Italy which also includes the highest rate of ignored patients. French patients were by far the less likely to receive accurate information from their physician. In conclusion, an actual educational deficit has been found in the patients regarding antibiotic use. The physician is in the best position for correcting the deficit. By implicating more the patients in the medical decision, he or she will deflate the ignored category, the most likely to misuse antibiotics, and hence to produce antibiotic resistance. PMID:15918656

  17. Blood transfusion: patient identification and empowerment.

    PubMed

    Stout, Lynn; Joseph, Sundari

    Positive patient identification is pivotal to several steps of the transfusion process; it is integral to ensuring that the correct blood is given to the correct patient. If patient misidentification occurs, this has potentially fatal consequences for patients. Historically patient involvement in healthcare has focused on clinical decision making, where the patient, having been provided with medical information, is encouraged to become involved in the decisions related to their individualised treatment. This article explores the aspects of patient contribution to patient safety relating to positive patient identification in transfusion. When involving patients in their care, however, clinicians must recognise the diversity of patients and the capacity of the patient to be involved. It must not be assumed that all patients will be willing or indeed able to participate. Additionally, clinicians' attitudes to patient involvement in patient safety can determine whether cultural change is successful. PMID:26878405

  18. Patient preference: a comparison of electronic patient-completed questionnaires with paper among cancer patients.

    PubMed

    Martin, P; Brown, M C; Espin-Garcia, O; Cuffe, S; Pringle, D; Mahler, M; Villeneuve, J; Niu, C; Charow, R; Lam, C; Shani, R M; Hon, H; Otsuka, M; Xu, W; Alibhai, S; Jenkinson, J; Liu, G

    2016-03-01

    In this study, we compared cancer patients preference for computerised (tablet/web-based) surveys versus paper. We also assessed whether the understanding of a cancer-related topic, pharmacogenomics is affected by the survey format, and examined differences in demographic and medical characteristics which may affect patient preference and understanding. Three hundred and four cancer patients completed a tablet-administered survey and another 153 patients completed a paper-based survey. Patients who participated in the tablet survey were questioned regarding their preference for survey format administration (paper, tablet and web-based). Understanding was assessed with a 'direct' method, by asking patients to assess their understanding of genetic testing, and with a 'composite' score. Patients preferred administration with tablet (71%) compared with web-based (12%) and paper (17%). Patients <65 years old, non-Caucasians and white-collar professionals significantly preferred the computerised format following multivariate analysis. There was no significant difference in understanding between the paper and tablet survey with direct questioning or composite score. Age (<65 years) and white-collar professionals were associated with increased understanding (both P = 0.03). There was no significant difference in understanding between the tablet and print survey in a multivariate analysis. Patients overwhelmingly preferred computerised surveys and understanding of pharmacogenomics was not affected by survey format. PMID:25899560

  19. [Preoperative structured patient education].

    PubMed

    Lamarche, D

    1993-04-01

    This article describes the factors that motivated the nursing staff of the cardiac surgery unit at the Royal Victoria Hospital in Montreal, to revise their preoperative teaching program. The motivating factors described are the length of the preoperative waiting period; the level of preoperative anxiety; the decreased length of hospital stay; the dissatisfaction of the nursing staff with current patient teaching practices; and the lack of available resources. The reorganization of the teaching program was based upon the previously described factors combined with a review of the literature that demonstrated the impact of preoperative anxiety, emotional support and psycho-educational interventions upon the client's recovery. The goals of the new teaching program are to provide the client and the family with cognitive and sensory information about the client's impending hospitalization, chronic illness and necessary lifestyle modifications. The program consists of a system of telephone calls during the preoperative waiting period; a videotape viewing; a tour of the cardiac surgery unit; informal discussion groups; and the availability of nursing consultation to decrease preoperative anxiety. The end result of these interventions is more time for client support and integration of necessary information by the client and family. This kind of program has the potential to provide satisfaction at many levels by identifying client's at risk; increasing client knowledge; increasing support; decreasing anxiety during the preoperative waiting period; and decreasing the length of hospital stay. The nursing staff gained a heightened sense of accomplishment because the program was developed according to the nursing department's philosophy, which includes primary nursing.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8472243

  20. Psychodynamics in medically ill patients.

    PubMed

    Nash, Sara Siris; Kent, Laura K; Muskin, Philip R

    2009-01-01

    This article explores the role of psychodynamics as it applies to the understanding and treatment of medically ill patients in the consultation-liaison psychiatry setting. It provides historical background that spans the eras from Antiquity (Hippocrates and Galen) to nineteenth-century studies of hysteria (Charcot, Janet, and Freud) and into the twentieth century (Flanders Dunbar, Alexander, Engle, and the DSM). The article then discusses the effects of personality on medical illness, treatment, and patients' ability to cope by reviewing the works of Bibring, Kahana, and others. The important contribution of attachment theory is reviewed as it pertains the patient-physician relationship and the health behavior of physically ill patients. A discussion of conversion disorder is offered as an example of psychodynamics in action. This article highlights the important impact of countertransference, especially in terms of how it relates to patients who are extremely difficult and "hateful," and explores the dynamics surrounding the topic of physician-assisted suicide, as it pertains to the understanding of a patient's request to die. Some attention is also given to the challenges surrounding the unique experience of residents learning how to treat medically ill patients on the consultation-liaison service. Ultimately, this article concludes that the use and understanding of psychodynamics and psychodynamic theory allows consultation-liaison psychiatrists the opportunity to interpret the life narratives of medically ill patients in a meaningful way that contributes importantly to treatment. PMID:19968453

  1. Hospitalization of the Suicidal Patient.

    ERIC Educational Resources Information Center

    Goldblatt, Mark J.

    1994-01-01

    Reviews development of safe treatment plans for suicidal patients in context of the psychodynamic formulation and therapeutic alliance. Suggests that decision to hospitalize is based upon patient issues: diagnosis, support system, history, and current stressors. Recommends that clinician err on side of conservative management with early…

  2. Patient Disclosure of Medical Misdeeds

    ERIC Educational Resources Information Center

    Bergen, Clara; Stivers, Tanya

    2013-01-01

    Modern patients walk a tightrope between respecting medical authority and acting as knowledgeable advocates regarding health issues, with the agency and responsibilities that come with this. This article uses conversation analysis to explore this balance in relation to patient disclosures of medical misdeeds in video-recorded primary care medical…

  3. Viscoelastic cushion for patient support

    NASA Technical Reports Server (NTRS)

    Sauers, D. G.

    1971-01-01

    Flexible container, filled with liquid, provides supportive device which conforms to patient's anatomy. Uniform cushion pressure prevents formation of decubitus ulcers, while the porous sponge substructure damps fluid movement through cushion response so that patient is not dumped when his weight shifts.

  4. Inflatable stretcher to transport patients

    NASA Technical Reports Server (NTRS)

    Clark, C. C.; Gordon, F. T., Jr.; Schmidt, C. B.

    1970-01-01

    Inflatable plastic bag inside strong, inflexible outer bag facilitates emergency transport of seriously burned or disabled patients. When the bag is inflated the patient is completely immobilized and cushioned from external shock. Air for breathing, temperature controls and communications may be provided by appropriate plug-in connections.

  5. Biotelemetry system for ambulatory patients

    NASA Technical Reports Server (NTRS)

    Fryer, T. B.

    1978-01-01

    Compact transmitter for multichannel telemetry of medical data is carried in patient's belt. Pulse-code modulation (PCM), is used for high-quality signal, and low-power CMOS integrated circuits make miniaturization possible. Transmitter is useful for electro-encephalograms (EEG) and electro-cardiograms (EKG) and other biomedical patient-monitoring situations.

  6. Preventing pitfalls in patient surveys.

    PubMed

    Steiber, S R

    1989-05-01

    Properly conceived, customer satisfaction surveys can yield the quantitative data needed to gauge patient satisfaction. But, as the author notes, these surveys can be "a veritable mine field of surprises for the uninitiated." This article, the last in a three-part series on measuring patient satisfaction, describes potential pitfalls and discusses the merits of in-person, mail and telephone surveys. PMID:10293191

  7. Tube feeding patients with dementia.

    PubMed

    Chernoff, Ronni

    2006-04-01

    As the population ages, the incidence of dementia increases. All types of dementia, whether they are reversible or irreversible, lead to loss of intellectual function and judgment, memory impairment, and personality changes. The skills to feed oneself, use eating utensils, and consume items recognized as food, thereby maintaining nutrition status, may be lost as dementia progresses. Reports indicate that nutrition status may be maintained when patients are hand fed, but this is labor intensive and therefore expensive. Feeding via a percutaneous endoscopic gastrostomy tube is often chosen as an acceptable alternative. Research indicates that there is little benefit in this population when aggressive nutrition support is instituted. Providing tube feeding to patients with dementia does not necessarily extend life, increase weight, or reduce the incidence of pressure ulcers or aspiration. There are many legal and ethical issues involved in the decision to place a feeding tube in demented patients. The primary issue in patients with dementia may be autonomy and the right of an individual to decide whether or not a tube should be placed at all. Legally, there is clear precedent that the courts see the insertion of a feeding tube as extraordinary care that the patient has the right to refuse. However, much of case law is derived from cases of patients who were in a persistent vegetative state. Advance directives help to determine what the patient would want for himself. Considering all the options before the patient can no longer make decisions is the most desirable course. PMID:16556924

  8. The Coronary Patient in Industry

    NASA Technical Reports Server (NTRS)

    Schuster, B.

    1971-01-01

    The coronary patient, as he pertains to industry particularly NASA, is discussed. Concepts of precoronary care, acute attacks which may develop while on the job, and the return of the cardiac patient to work are covered. Major emphasis was on the prevention of sudden death due to coronary disease.

  9. Pharmacotherapeutics for the AIDS Patient.

    ERIC Educational Resources Information Center

    Fife, Kenneth H.

    1991-01-01

    Anticipated shifts in the demographics of the Acquired Immune Deficiency Syndrome (AIDS) epidemic are examined, current state-of-the-art AIDS patient management is summarized, and some unique facets of drug therapy in the AIDS patient are discussed, including adverse reactions, complex drug interactions, use of investigational drugs, and…

  10. Optimal Nutrition in Hemodialysis Patients

    PubMed Central

    Ikizler, T. Alp

    2012-01-01

    Protein energy wasting (PEW) is highly prevalent in patients undergoing maintenance hemodialysis (MHD) patients. Importantly, there is a robust association between the extent of PEW and the risk of hospitalization and death in these patients, regardless of the nutritional marker used. The multiple etiologies of PEW in advanced kidney disease are still being elucidated. Apart from the multiple mechanisms that might lead to PEW, it appears that the common pathway for all the derangements is related to exaggerated protein degradation along with decreased protein synthesis. The hemodialysis procedure per se is an important contributor to this process. Metabolic and hormonal derangements such as acidosis, inflammation and resistance to anabolic properties of insulin resistance and growth hormone are all implicated for the development of PEW in MHD patients. Appropriate management of MHD patients at risk for PEW requires a comprehensive combination of strategies to diminish protein and energy depletion, and to institute therapies that will avoid further losses. The mainstay of nutritional treatment in MHD patients is provision of an adequate amount of protein and energy, using oral supplementation as needed. Intradialytic parenteral nutrition should be attempted in patients who cannot use the gastrointestinal tract efficiently. Other anabolic strategies such as exercise, anabolic hormones, anti-inflammatory therapies and appetite stimulants can be considered as complementary therapies in suitable patients. PMID:23439378

  11. [Patient education and breast cancer].

    PubMed

    Arfé, Emmanuelle; Bombail, Marie

    2015-10-01

    The therapeutic education program set up at the Institut Universitaire du Cancer (University Cancer Institute) in Toulouse accompanies the treatment pathway of breast cancer patients. It includes nine collective workshops. From work organization to application, professionals and patients are closely involved. PMID:26455624

  12. Patient Education Workshop: Summary Report.

    ERIC Educational Resources Information Center

    Simmons, Jeannette

    This report describes activities of a workshop conducted for fourteen health education practitioners and administrators from a number of different hospital and health care settings and geographical regions to appraise the state of patient education programs. The initiation and growth of patient education programs in a variety of health care…

  13. Communicating effectively with deaf patients.

    PubMed

    McAleer, Monica

    This article explores the communication needs of deaf patients who use British Sign Language as their first or preferred language. It would appear that these needs are not being met, particularly in acute hospital settings. Practical advice is provided for nurses to improve the quality of care that deaf patients receive. PMID:16438331

  14. Communicating with the deaf patient.

    PubMed

    Ekstrom, I

    1994-01-01

    Three levels of deafness exist: persons who are born deaf, persons who lost hearing after speech was acquired, and persons with sudden deafness. Nurses need to find methods to communicate with the patient, and not the family in lieu of the patient. Props, audio-visual aids, gestures, and written information should be used. PMID:7800759

  15. Patient Disclosure of Medical Misdeeds

    ERIC Educational Resources Information Center

    Bergen, Clara; Stivers, Tanya

    2013-01-01

    Modern patients walk a tightrope between respecting medical authority and acting as knowledgeable advocates regarding health issues, with the agency and responsibilities that come with this. This article uses conversation analysis to explore this balance in relation to patient disclosures of medical misdeeds in video-recorded primary care medical

  16. Virtual Patients in Geriatric Education

    ERIC Educational Resources Information Center

    Tan, Zaldy S.; Mulhausen, Paul L.; Smith, Stephen R.; Ruiz, Jorge G.

    2010-01-01

    The virtual patient is a case-based computer program that combines textual information with multimedia elements such as audio, graphics, and animation. It is increasingly being utilized as a teaching modality by medical educators in various fields of instruction. The inherent complexity of older patients and the shortage of geriatrics educators…

  17. Patient With Borderline Personality Disorder

    PubMed Central

    Griffiths, Dorothy E.

    1989-01-01

    Every family practice includes people who are difficult to manage. Persons with a borderline personality disorder can be the most difficult of all. They will trust no one, and consequently few, if any, others will be able to tolerate their profoundly difficult interpersonal communication style. These patients will present to their family physician more and more often with a variety of somatic and emotional symptoms. They will demand, either verbally or silently, that these symptoms be relieved immediately. This increasing demand for immediate response may eventually cause the physician to reject the patient. An understanding of this condition and how it develops in infancy may enable the physician to help the patient. A family physician who can set appropriate limits to the patient's demands may slowly convince the patient that he can trust and not be hurt. PMID:21248944

  18. Treating older patients with AML

    PubMed Central

    Amadori, S

    2012-01-01

    The treatment of older patients with acute myeloid leukemia is a difficult challenge. Older adults are more likely to have comorbidities that can limit treatment options, the disease tends to be more aggressive biologically and outcomes are worse than those in younger patients. Deciding which older patients would benefit from intensive chemotherapy is difficult, and efforts are underway to improve existing risk-assessment tools. Treatment should be individualized and may include standard chemotherapy for those patients who have none or at most one adverse factor, or investigational agents for those presenting with multiple poor-risk features. Low-intensity therapies are recommended for those patients who are deemed too frail to tolerate myelosuppressive regimens.

  19. Discussing cardiopulmonary resuscitation with patients.

    PubMed

    Attwood, S; Anderson, K; Mitchell, T

    The British Medical Association and the Royal College of Nursing acknowledge that patients should be involved in the decision-making process regarding cardiopulmonary resuscitation (CPR) in order to gain compliance with any decision made on their behalf. In the past, it was apparent that decisions concerning the eligibility of patients for CPR were being made arbitrarily and older people were being treated unfairly in respect of their consideration for this intervention. This article reviews literature associated with patient involvement in decision-making regarding CPR and 'do not resuscitate' orders and how an assessment and rehabilitation unit promoted this activity as usual practice. In order to enhance partnership with patients and promote best possible practice in respect of CPR decision making the authors suggest a strategy that includes (1) evaluating documentation (2) development of a patient information leaflet and (3) an education programme for healthcare personnel. PMID:11893959

  20. 'The patient': at the center of patient-reported outcomes.

    PubMed

    Awad, A George

    2015-01-01

    The recent emphasis of including patient reports in their own care management is reviewed in terms of the factors that contributed to its popularity. The role change of patients as being active participants in their own care as a result of the rising consumerism and advocacy has led to increased pressures for including patients in the therapeutic decision-making process. As consumers of clinical services, their perspectives and attitudes towards health and illness acquired more importance. The rising cost of healthcare has added another dimension in cost containment by empowering patients and sharing responsibility in their recovery, which hopefully can improve outcomes. Challenges in the development and implementation of patient-reported outcomes in psychiatry are reviewed and include the still unresolved subjective/objective dichotomy, identification of the most appropriate and relevant patient-reported outcomes. Few outcomes are identified and include: subjective tolerability of medication, self-reported health-related quality of life, preferences, patients' attitudes towards health and illness, satisfaction with medication and overall satisfaction of quality of care, and functional state, with particular focus on social functioning. PMID:26289737

  1. Patients' feelings about hand washing, MRSA status and patient information.

    PubMed

    Duncan, Christopher Paul; Dealey, Carol

    The purpose of this study was to explore patient opinion about asking healthcare professionals to wash their hands before a clinical procedure and to explore if methicillin-resistant Staphylococcus aureus (MRSA) status and access to patient information about infection control would influence the patients' anxiety about asking. A descriptive survey was undertaken using a semi-structured questionnaire. The questionnaire was distributed to a randomized convenience sample of 185 inpatients across all departments of an acute NHS Trust hospital (response rate 58.9%). Spearman's rank order and Kendall Tau-b tests were used to analyse specific correlations. Respondents were more confident than anxious about being involved in a campaign that empowered patients to ask staff to wash their hands. Patients were more anxious to ask if their previous admission episodes were fewer, if their knowledge of MRSA was high and if there was less information about infection control available. Patients who had contracted MRSA in the past were less anxious, as they had a better understanding of the disease. In addition, more patients felt less anxious about asking staff to wash their hands if staff wore a badge saying 'It's OK to ask'. PMID:17353833

  2. Do patient assessments of primary care differ by patient ethnicity?

    PubMed Central

    Taira, D A; Safran, D G; Seto, T B; Rogers, W H; Inui, T S; Montgomery, J; Tarlov, A R

    2001-01-01

    OBJECTIVE: To determine if patient assessments (reports and ratings) of primary care differ by patient ethnicity. DATA SOURCES/STUDY DESIGN: A self-administered patient survey of 6,092 Massachusetts employees measured seven defining characteristics of primary care: (1) access (financial, organizational); (2) continuity (longitudinal, visit based); (3) comprehensiveness (knowledge of patient, preventive counseling); (4) integration; (5) clinical interaction (communication, thoroughness of physical examinations); (6) interpersonal treatment; and (7) trust. The study employed a cross-sectional observational design. PRINCIPAL FINDINGS: Asians had the lowest primary care performance assessments of any ethnic group after adjustment for socioeconomic and other factors. For example, compared to whites, Asians had lower scores for communication (69 vs. 79, p = .001) and comprehensive knowledge of patient (56 vs. 48, p = .002), African Americans and Latinos had less access to care, and African Americans had less longitudinal continuity than whites. CONCLUSIONS: We do not know what accounts for the observed differences in patient assessments of primary care. The fact that patient reports as well as the more subjective ratings of care differed by ethnicity suggests that quality differences might exist that need to be addressed. PMID:11775667

  3. Complement activity is associated with disease severity in multifocal motor neuropathy

    PubMed Central

    Vlam, Lotte; Cats, Elisabeth A.; Harschnitz, Oliver; Jansen, Marc D.; Piepers, Sanne; Veldink, Jan Herman; Franssen, Hessel; Stork, Abraham C.J.; Heezius, Erik; Rooijakkers, Suzan H.M.; Herpers, Bjorn L.; van Strijp, Jos A.; van den Berg, Leonard H.

    2015-01-01

    Objective: To investigate whether high innate activity of the classical and lectin pathways of complement is associated with multifocal motor neuropathy (MMN) and whether levels of innate complement activity or the potential of anti-GM1 antibodies to activate the complement system correlate with disease severity. Methods: We performed a case-control study including 79 patients with MMN and 79 matched healthy controls. Muscle weakness was documented with Medical Research Council scale sum score and axonal loss with nerve conduction studies. Activity of the classical and lectin pathways of complement was assessed by ELISA. We also determined serum mannose-binding lectin (MBL) concentrations and polymorphisms in the MBL gene (MBL2) and quantified complement-activating properties of anti-GM1 IgM antibodies by ELISA. Results: Activity of the classical and lectin pathways, MBL2 genotypes, and serum MBL concentrations did not differ between patients and controls. Complement activation by anti-GM1 IgM antibodies was exclusively mediated through the classical pathway and correlated with antibody titers (p < 0.001). Logistic regression analysis showed that both high innate activity of the classical pathway of complement and high complement-activating capacity of anti-GM1 IgM antibodies were significantly associated with more severe muscle weakness and axonal loss. Conclusion: High innate activity of the classical pathway of complement and efficient complement-activating properties of anti-GM1 IgM antibodies are determinants of disease severity in patients with MMN. These findings underline the importance of anti-GM1 antibody–mediated complement activation in the pathogenesis and clinical course of MMN. PMID:26161430

  4. Patient Admission Preferences and Perceptions

    PubMed Central

    Wu, Clayton; Melnikow, Joy; Dinh, Tu; Holmes, James F.; Gaona, Samuel D.; Bottyan, Thomas; Paterniti, Debora; Nishijima, Daniel K.

    2015-01-01

    Introduction Understanding patient perceptions and preferences of hospital care is important to improve patients’ hospitalization experiences and satisfaction. The objective of this study was to investigate patient preferences and perceptions of hospital care, specifically differences between intensive care unit (ICU) and hospital floor admissions. Methods This was a cross-sectional survey of emergency department (ED) patients who were presented with a hypothetical scenario of a patient with mild traumatic brain injury (TBI). We surveyed their preferences and perceptions of hospital care related to this scenario. A closed-ended questionnaire provided quantitative data on patient preferences and perceptions of hospital care and an open-ended questionnaire evaluated factors that may not have been captured with the closed-ended questionnaire. Results Out of 302 study patients, the ability for family and friends to visit (83%), nurse availability (80%), and physician availability (79%) were the factors most commonly rated “very important,” while the cost of hospitalization (62%) and length of hospitalization (59%) were the factors least commonly rated “very important.” When asked to choose between the ICU and the floor if they were the patient in the scenario, 33 patients (10.9%) choose the ICU, 133 chose the floor (44.0%), and 136 (45.0%) had no preference. Conclusion Based on a hypothetical scenario of mild TBI, the majority of patients preferred admission to the floor or had no preference compared to admission to the ICU. Humanistic factors such as the availability of doctors and nurses and the ability to interact with family appear to have a greater priority than systematic factors of hospitalization, such as length and cost of hospitalization or length of time in the ED waiting for an in-patient bed. PMID:26587095

  5. Impact of Physician–Patient Discussions on Patient Satisfaction

    PubMed Central

    Chen, Judy Y.; Tao, May L.; Tisnado, Diana; Malin, Jennifer; Ko, Clifford; Timmer, Martha; Adams, John L.; Ganz, Patricia A.; Kahn, Katherine L.

    2010-01-01

    Background When 2 treatment choices (ie, mastectomy vs. breast conserving therapy) show no difference in a primary clinical outcome (ie, survival), patient satisfaction becomes an important marker of the quality of care received. Objectives To assess the impact of physician–patient discussion of primary surgical treatment outcomes on patients’ satisfaction with medical care (MC) among women with incident breast cancer (BC). Method We used self-report data of a population-based survey of 495 women ≥50 years of age with stage I–II BC in Los Angeles, California in 2000 conducted a mean of 7.5 and 24 months after diagnosis. Using multivariable analyses, we evaluated the impact of physician–patient outcome discussions (ie, BC recurrence, BC survival, breast appearance, and arm swelling/pain/movement difficulty) on patient satisfaction at baseline and follow-up. Results Most women were satisfied with their MC (>65%). More than half reported physician–patient discussions of BC recurrence (54%), breast appearance (50%), and arm pain/swelling/movement difficulty (55%). Thirty-one percent discussed BC survival. Women who discussed arm swelling, pain, movement difficulty were significantly more likely to be satisfied at baseline (odds ratio: 1.8, 95% confidence interval: 1.1–3.0, P < 0.05) and follow-up (odds ratio: 1.9, 95% confidence interval: 1.2–3.0, P > 0.01). The more treatment outcomes patients discussed with their physicians, the higher patient satisfaction ratings were at baseline and follow-up. Conclusions Physician–patient discussions of BC treatment outcomes were highly correlated with patients’ satisfaction with overall MC regardless of the procedure received. This suggests that the quality of BC care should include assessments of physician–patient communication. PMID:18953226

  6. Mutation in GM2A Leads to a Progressive Chorea-dementia Syndrome

    PubMed Central

    Salih, Mustafa A.; Seidahmed, Mohammed Z.; El Khashab, Heba Y.; Hamad, Muddathir H. A.; Bosley, Thomas M.; Burn, Sabrina; Myers, Angela; Landsverk, Megan L.; Crotwell, Patricia L.; Bilguvar, Kaya; Mane, Shrikant; Kruer, Michael C.

    2015-01-01

    Background The etiology of many cases of childhood-onset chorea remains undetermined, although advances in genomics are revealing both new disease-associated genes and variant phenotypes associated with known genes. Methods We report a Saudi family with a neurodegenerative course dominated by progressive chorea and dementia in whom we performed homozygosity mapping and whole exome sequencing. Results We identified a homozygous missense mutation in GM2A within a prominent block of homozygosity. This mutation is predicted to impair protein function. Discussion Although discovered more than two decades ago, to date, only five patients with this rare form of GM2 gangliosidosis have been reported. The phenotype of previously described GM2A patients has been typified by onset in infancy, profound hypotonia and impaired volitional movement, intractable seizures, hyperacusis, and a macular cherry red spot. Our findings expand the phenotypic spectrum of GM2A mutation-positive gangliosidosis to include generalized chorea without macular findings or hyperacusis and highlight how mutations in neurodegenerative disease genes may present in unexpected ways. PMID:26203402

  7. [Preanesthetic evaluation of pediatric patients].

    PubMed

    Nishina, Kahoru; Maekawa, Nobuhiro

    2010-09-01

    Preoperative evaluation of pediatric patients undergoing anesthesia requires specific consideration. Upper respiratory infection and fever are common problems encountered in the otherwise healthy children who are scheduled for minor surgery. Considerations for the preanesthetic evaluation of the patients with asthma or ex-preterm infants are discussed. It is important to provide less stressful environment to patients and to fulfill patients' satisfaction. Fasting time should be minimum. If patients are extremely anxious and feel fear for separation from their parents, anxiolytic medication might be helpful. Parents present at induction may be another option for smooth induction of anesthesia. Children may have received vaccines. Surgery should be planned in accordance with the vaccination program. Several laboratory examinations can be omitted after careful physical examination and interview. Blood count, ECG, or chest X-ray seldom affect the preoperative preparation or anesthetic method for the patients who do not have any particular findings in physical examination. Such examination is not only unnecessary, but gives stress to those patients. In recent years, there have been some reports that anesthesia during infancy affect the development of the brain and causes learning disability or emotional complication. These findings are mainly based on the results of animal experiments and the effect of anesthetics on the developing brain has not been thoroughly confirmed yet in the clinical settings. We need to keep our eyes on the forthcoming research. PMID:20857667

  8. Confabulations in alcoholic Korsakoff patients.

    PubMed

    Borsutzky, Sabine; Fujiwara, Esther; Brand, Matthias; Markowitsch, Hans J

    2008-11-01

    Besides forgetting, memory is also prone to distortions, errors and illusions. Confabulation is one type of memory distortion that may occur in cases of brain damage. Although confabulations are described anecdotally in patients with alcoholic Korsakoff syndrome (KS), there are few systematic investigations of the presence and nature of these types of false memories in KS. Moreover, it is unclear whether KS patients' confabulations evenly affect all types of memories, or whether certain memory domains are more susceptible. Our study attempted to clarify two questions: first, whether confabulations are a critical feature of the cognitive impairment associated with long-term KS in a large sample of patients (N=42). Second, we investigated which memory domain is most likely affected by confabulations in KS. To elicit confabulations, we used a Confabulation Interview containing questions from different memory domains. We found that KS patients overall confabulated more compared to a group of healthy subjects. Furthermore, we found that patients confabulated most within the episodic/autobiographical memory domain. Our results imply that besides pronounced memory deficits typically associated with KS, confabulation can also be regarded as a clinical feature of the disease. The preponderance of episodic confabulation obtained here by using a standardized test, confirms anecdotic reports that KS patients confabulate in everyday life mainly with respect to their personal past and present. Thus, for a detailed description of the memory profile of KS patients, the screening of confabulation tendencies may be a useful supplementary clinical tool. PMID:18675286

  9. [Characteristic features of gouty patients].

    PubMed

    Nakamura, T; Tsutani, H; Ueda, T

    1996-12-01

    Characteristic feature of pathogenesis, epidemiology and laboratory findings in hyperuricemia of gouty patients are studied and reasonable treatments of gout in clinical medicine are discussed. Gout is characterized by repeated arthritis attacks on the metacarpophalangeal joint of the first toe or other small joints, especially overworked joints or those exposed to cold. The arthritis attack lasts for 3.5 days and then diminishes gradually. The intervals are shortened in patients under poor hyperuricemic control but tophi formation is less frequent. Complications in combination with hyperlipidemia, diabetes mellitus, obesity and hypertension, which are compatible to syndrome X, are frequent in gouty patients and are suspected of rapidly progressing to arteriosclerosis, such as ischemic heart diseases. Hyperuricemia consists of over-production and underexcretion, which can be diagnosed by the urate clearance test. Classification is valuable for surveying the underlying diseases of secondary hyperuricemia and treating gouty patients. Underexcretion was observed in 85% of gouty patients with hyperuricemia and even the mean urate clearance in the overproduction type was significantly lower than that of normal controls, suggesting that underexcretion is a fundamental phenomenon in all gouty patients. Treatments of complications as well as those of hyperuricemia with uricosuric agents are required for clinical treatment of gouty patients. PMID:8976100

  10. Methadone patients and alcohol abuse.

    PubMed

    Ottomanelli, G

    1999-03-01

    A literature review was conducted on the excess use of alcohol by methadone patients. Although the rate of alcohol abuse among methadone patients was found to be high (compared to general population estimates), the rate was comparable to individuals who engaged in risk-taking behaviors and individuals under stress, such as college students and emergency room patients. Comparisons of rates for different samples was difficult because of the varying criteria used to define alcohol abuse and the absence of operational criteria applicable across different populations. Another source of difficulty was that some studies evaluated motivational variables (why the person drank) and found them to be more important predictors than sociodemographic variables (for example, history of drinking and social class). Contrary to the expectation that methadone patients who consumed excessive amounts of alcohol would require higher dosages of methadone, it was found that nonalcohol-abusing methadone patients requested the higher dosage levels. This finding, however, was subject to methodological confounds. The issue of whether the methadone patient who abused alcohol has a negative treatment outcome was a multifaceted question. The definition of treatment outcome for alcohol-abusing methadone patients determined whether the investigator concluded that there was a negative impact versus minimal impact. PMID:10023608

  11. Patient satisfaction: focusing on "excellent".

    PubMed

    Otani, Koichiro; Waterman, Brian; Faulkner, Kelly M; Boslaugh, Sarah; Burroughs, Thomas E; Dunagan, W Claiborne

    2009-01-01

    In an emerging competitive market such as healthcare, managers should focus on achieving excellent ratings to distinguish their organization from others. When it comes to customer loyalty, "excellent" has a different meaning. Customers who are merely satisfied often do not come back. The purpose of this study was to find out what influences adult patients to rate their overall experience as "excellent." The study used patient satisfaction data collected from one major academic hospital and four community hospitals. After conducting a multiple logistic regression analysis, certain attributes were shown to be more likely than others to influence patients to rate their experiences as excellent. The study revealed that staff care is the most influential attribute, followed by nursing care. These two attributes are distinctively stronger drivers of overall satisfaction than are the other attributes studied (i.e., physician care, admission process, room, and food). Staff care and nursing care are under the control of healthcare managers. If improvements are needed, they can be accomplished through training programs such as total quality management or continuous quality improvement, through which staff employees and nurses learn to be sensitive to patients' needs. Satisfying patients' needs is the first step toward having loyal patients, so hospitals that strive to ensure their patients are completely satisfied are more likely to prosper. PMID:19413164

  12. [Patients' increasing role in healthcare].

    PubMed

    Colombo, Cinzia

    2016-03-01

    Since the late '90s citizens have been increasingly involved in healthcare, thanks to a shift in access to healthcare information, greater patients' and citizens' awareness about their rights and needs, and a change in physicians' attitudes and behavior. At the same time, to ensure the sustainability of the national health services, patients' needs are increasingly being examined in relation to the services and interventions needed, and to foster informed demand by patients and citizens, and appropriate prescriptions by physicians. Nowadays, patients already have a section in an authoritative medical journal. Working with clinicians, they set research priorities and the outcomes to be studied. Especially in UK and USA, they are invited to participate in the design and development of trials and in deciding which trials should be funded. The situation varies widely in different countries, though involving citizens in healthcare decisions is a common mantra. Even when they consult a clinician, the patients' role has evolved. People ask for information more often and want an active part in decisions about their health. Physicians start to pay more attention to the person and the social context, feelings and emotions, and person-centered care has become a reference in the doctor-patient relationship. This article offers an overview of the changing roles of patients and citizens in healthcare. PMID:27030220

  13. Transport of the trauma patient.

    PubMed

    Davies, G; Chesters, A

    2015-07-01

    The transport of the seriously injured patient is associated with risk and requires particular expertise and attention. The aim of this review is to provide a historical overview of transport services available to trauma patients in the UK, describe the various transport platforms that are used, identify risks from a system and disease perspective and how they may be mitigated, and make international comparisons. The transfer of patients requiring medical attention has developed over the years and now includes complex undertakings that undoubtedly confer a degree of risk on the patient. A number of different transport platforms are in regular use in the UK, and a number of different health-care professions of varying training, experience, and seniority undertake these transfers. The general principles are to provide no worse care en route than has been provided at the departure destination and to transport patients to a destination capable of delivering whichever intervention the patient is deemed to require. When deciding to transport an injured patient, there are risks, and appropriate mitigation must be in place, particularly if primary transfer to a major trauma centre involves bypassing a nearer facility. It is clear that those clinicians who undertake medical transfers must be appropriately trained and must have access to local or national guidelines. Medical transfers must be the subject of ongoing research, both to ensure that best practice is in place and to continue to understand the safest way of achieving essential transfers effectively. PMID:26089445

  14. Access ligation in transplant patients.

    PubMed

    Wilmink, Teun; Hollingworth, Lee; Dasgupta, Indranil

    2016-03-01

    Access surgeons will encounter patients with functioning transplants who want to lose their fistula, and every dialysis unit sees patients returning after a failed kidney transplant for whom an old fistula is a readily available lifeline. The decision is straightforward in patients with perfectly functioning transplants and disabling complications of their fistula, or in patients with failing transplants and a good fistula. The challenge is to make this decision in patients with good transplant function and an asymptomatic fistula. Despite improvements in one-year survival of renal grafts, the long-term graft survival has improved modestly. This means about half of the patients with a successful kidney transplant will return to dialysis within 10 years. Use of recently developed risk calculators, based on clinical parameters, may help in the decision process. A high flow fistula can lead to heart failure but most fistulae are well tolerated in asymptomatic patients and the effects of closure of the AVF on the heart are modest. Recent evidence suggests that there may be benefits of a functioning AVF that may need to be considered in this decision process. This article reviews the literature and comes to pragmatic recommendations of what to do with this conundrum. PMID:26951908

  15. HPV Carcinomas in Immunocompromised Patients

    PubMed Central

    Reusser, Nicole M.; Downing, Christopher; Guidry, Jacqueline; Tyring, Stephen K.

    2015-01-01

    Human papillomavirus (HPV) infection is the most common sexually transmitted disease worldwide and can result in pre-malignancies or overt malignancies of the skin and mucosal surfaces. HPV-related illnesses are an important personal and public health problem causing physical, mental, sexual and financial detriments. Moreover, this set of malignancies severely affects the immunosuppressed population, particularly HIV-positive patients and organ-transplant recipients. There is growing incidence of HPV-associated anogenital malignancies as well as a decrease in the average age of affected patients, likely related to the rising number of high-risk individuals. Squamous cell carcinoma is the most common type of HPV-related malignancy. Current treatment options for HPV infection and subsequent disease manifestations include imiquimod, retinoids, intralesional bleomycin, and cidofovir; however, primary prevention with HPV vaccination remains the most effective strategy. This review will discuss anogenital lesions in immunocompromised patients, cutaneous warts at nongenital sites, the association of HPV with skin cancer in immunocompromised patients, warts and carcinomas in organ-transplant patients, HIV-positive patients with HPV infections, and the management of cutaneous disease in the immunocompromised patient. PMID:26239127

  16. HPV Carcinomas in Immunocompromised Patients.

    PubMed

    Reusser, Nicole M; Downing, Christopher; Guidry, Jacqueline; Tyring, Stephen K

    2015-01-01

    Human papillomavirus (HPV) infection is the most common sexually transmitted disease worldwide and can result in pre-malignancies or overt malignancies of the skin and mucosal surfaces. HPV-related illnesses are an important personal and public health problem causing physical, mental, sexual and financial detriments. Moreover, this set of malignancies severely affects the immunosuppressed population, particularly HIV-positive patients and organ-transplant recipients. There is growing incidence of HPV-associated anogenital malignancies as well as a decrease in the average age of affected patients, likely related to the rising number of high-risk individuals. Squamous cell carcinoma is the most common type of HPV-related malignancy. Current treatment options for HPV infection and subsequent disease manifestations include imiquimod, retinoids, intralesional bleomycin, and cidofovir; however, primary prevention with HPV vaccination remains the most effective strategy. This review will discuss anogenital lesions in immunocompromised patients, cutaneous warts at nongenital sites, the association of HPV with skin cancer in immunocompromised patients, warts and carcinomas in organ-transplant patients, HIV-positive patients with HPV infections, and the management of cutaneous disease in the immunocompromised patient. PMID:26239127

  17. Celebrity Patients, VIPs, and Potentates

    PubMed Central

    Groves, James E.; Dunderdale, Barbara A.; Stern, Theodore A.

    2002-01-01

    Background: During the second half of the 20th century, the literature on the doctor-patient relationship mainly dealt with the management of “difficult” (personality-disordered) patients. Similar problems, however, surround other types of “special” patients. Method: An overview and analysis of the literature were conducted. As a result, such patients can be subcategorized by their main presentations; each requires a specific management strategy. Results: Three types of “special” patients stir up irrational feelings in their caregivers. Sick celebrities threaten to focus public scrutiny on the private world of medical caregivers. VIPs generate awe in caregivers, with loss of the objectivity essential to the practice of scientific medicine. Potentates unearth narcissism in the caregiver-patient relationship, which triggers a struggle between power and shame. Pride, privacy, and the staff's need to be in control are all threatened by introduction of the special patient into medicine's closed culture. Conclusion: The privacy that is owed to sick celebrities should be extended to protect overexposed staff. The awe and loss of medical objectivity that VIPs generate are counteracted by team leadership dedicated to avoiding any deviation from standard clinical procedure. Moreover, the collective ill will surrounding potentates can be neutralized by reassuring them that they are “special”—and by caregivers mending their own vulnerable self-esteem. PMID:15014712

  18. Interactive Visualization for Patient-to-Patient Comparison.

    PubMed

    Nguyen, Quang Vinh; Nelmes, Guy; Huang, Mao Lin; Simoff, Simeon; Catchpoole, Daniel

    2014-03-01

    A visual analysis approach and the developed supporting technology provide a comprehensive solution for analyzing large and complex integrated genomic and biomedical data. This paper presents a methodology that is implemented as an interactive visual analysis technology for extracting knowledge from complex genetic and clinical data and then visualizing it in a meaningful and interpretable way. By synergizing the domain knowledge into development and analysis processes, we have developed a comprehensive tool that supports a seamless patient-to-patient analysis, from an overview of the patient population in the similarity space to the detailed views of genes. The system consists of multiple components enabling the complete analysis process, including data mining, interactive visualization, analytical views, and gene comparison. We demonstrate our approach with medical scientists on a case study of childhood cancer patients on how they use the tool to confirm existing hypotheses and to discover new scientific insights. PMID:24748858

  19. Interactive Visualization for Patient-to-Patient Comparison

    PubMed Central

    Nelmes, Guy; Huang, Mao Lin; Simoff, Simeon; Catchpoole, Daniel

    2014-01-01

    A visual analysis approach and the developed supporting technology provide a comprehensive solution for analyzing large and complex integrated genomic and biomedical data. This paper presents a methodology that is implemented as an interactive visual analysis technology for extracting knowledge from complex genetic and clinical data and then visualizing it in a meaningful and interpretable way. By synergizing the domain knowledge into development and analysis processes, we have developed a comprehensive tool that supports a seamless patient-to-patient analysis, from an overview of the patient population in the similarity space to the detailed views of genes. The system consists of multiple components enabling the complete analysis process, including data mining, interactive visualization, analytical views, and gene comparison. We demonstrate our approach with medical scientists on a case study of childhood cancer patients on how they use the tool to confirm existing hypotheses and to discover new scientific insights. PMID:24748858

  20. Mining time-dependent patient outcomes from hospital patient records.

    PubMed Central

    Rao, Bharat R.; Sandilya, Sathyakama; Niculescu, Radu; Germond, Colin; Goel, A.

    2002-01-01

    We describe REMIND, a data mining framework that accurately infers missing clinical information by reasoning over the entire patient record. Hospitals collect computerized patient records (CPR's) in structured (database tables) and unstructured (free text) formats. Structured clinical data in the CPR's is often poorly recorded, and information may be missing about key outcomes and processes. For instance, for a population of 344 colon cancer patients, important clinical outcomes, such as disease state and its evolution, are stored only as unstructured data (doctors' dictations) in the CPR. Raw evidence (extracted directly from the CPR) is not a good predictor of disease state. Yet by combining this evidence in a principled fashion (using methods from uncertain and temporal reasoning), REMIND accurately infers disease state sequences for recurrence, a complex time-varying outcome, for these patients. These outcomes can now be added back into the CPR in structured form. PMID:12463900

  1. Shader Lamps Virtual Patients: the physical manifestation of virtual patients.

    PubMed

    Rivera-Gutierrez, Diego; Welch, Greg; Lincoln, Peter; Whitton, Mary; Cendan, Juan; Chesnutt, David A; Fuchs, Henry; Lok, Benjamin

    2012-01-01

    We introduce the notion of Shader Lamps Virtual Patients (SLVP) - the combination of projector-based Shader Lamps Avatars and interactive virtual humans. This paradigm uses Shader Lamps Avatars technology to give a 3D physical presence to conversational virtual humans, improving their social interactivity and enabling them to share the physical space with the user. The paradigm scales naturally to multiple viewers, allowing for scenarios where an instructor and multiple students are involved in the training. We have developed a physical-virtual patient for medical students to conduct ophthalmic exams, in an interactive training experience. In this experience, the trainee practices multiple skills simultaneously, including using a surrogate optical instrument in front of a physical head, conversing with the patient about his fears, observing realistic head motion, and practicing patient safety. Here we present a prototype system and results from a preliminary formative evaluation of the system. PMID:22357021

  2. Patient information preferences among breast and prostate cancer patients.

    PubMed

    Sharpley, C F; Christie, D R H

    2007-04-01

    Preferences for information about their disease and treatment were collected from 392 patients who had been treated for either breast or prostate cancer an average of 2 years previously. Type of information that they had received, their ratings of its value to them and their preferred format for further information were examined. The most common and most preferred form of information was through doctor interview, followed by educational booklets. Prostate cancer patients preferred videotapes; breast cancer patients tended to prefer individualized approaches including a tour of the department. Effects of age, educational levels, occupational backgrounds and self-reports for anxiety and depression at the time of the survey and at time of diagnosis were analysed. Prostate cancer patients who were most severely depressed showed a preference for not receiving any information at all, perhaps reflecting a tendency towards withdrawal. PMID:17419861

  3. Quality of Doctor-Patient Communication through the Eyes of the Patient: Variation According to the Patient's Educational Level

    ERIC Educational Resources Information Center

    Aelbrecht, Karolien; Rimondini, Michela; Bensing, Jozien; Moretti, Francesca; Willems, Sara; Mazzi, Mariangela; Fletcher, Ian; Deveugele, Myriam

    2015-01-01

    Good doctor-patient communication may lead to better compliance, higher patient satisfaction, and finally, better health. Although the social variance in how physicians and patients communicate is clearly demonstrated, little is known about what patients with different educational attainments actually prefer in doctor-patient communication. In

  4. Quality of Doctor-Patient Communication through the Eyes of the Patient: Variation According to the Patient's Educational Level

    ERIC Educational Resources Information Center

    Aelbrecht, Karolien; Rimondini, Michela; Bensing, Jozien; Moretti, Francesca; Willems, Sara; Mazzi, Mariangela; Fletcher, Ian; Deveugele, Myriam

    2015-01-01

    Good doctor-patient communication may lead to better compliance, higher patient satisfaction, and finally, better health. Although the social variance in how physicians and patients communicate is clearly demonstrated, little is known about what patients with different educational attainments actually prefer in doctor-patient communication. In…

  5. Special Considerations in Trauma Patients.

    PubMed

    Abraham, Michael K; Aquino, Patrick R; Kuo, Dick C

    2015-11-01

    The emergent management of a traumatic injury can be an extremely intense situation. These assessments can be even more difficult when patients have an underlying psychiatric condition. After a protocoled evaluation of the traumatic injuries, the psychological manifestation of diseases can be addressed. The appropriate use of physical or chemical restraints to facilitate the work-up is paramount in the ability of the provider to protect patients and staff from agitated and traumatized patients. The emergency medicine provider should have a low threshold for including psychiatry in the treatment plans, as the long-term sequelae of these entities require specialized treatment. PMID:26493528

  6. Selection of Salvage Cryotherapy Patients

    PubMed Central

    Katz, Aaron E; Ghafar, Mohamed A

    2002-01-01

    Radiation therapy is a main form of therapy for patients with localized prostate cancer. Despite advances in delivering radiation beams to the gland, urologists will be faced with managing patients with rising prostate-specific antigen values and radiation-recurrent cancer. If the cancer is detected early, salvage therapy can be initiated. Recent modifications in the technique of salvage cryosurgery have led to the ability to eradicate these tumors with a decrease in morbidity. The management and selection of these patients, as well as the results of salvage cryoablation, are discussed in this article. PMID:16986007

  7. [The obese patient's therapeutic course].

    PubMed

    Giusti, V

    2006-03-29

    Obesity as a chronic disease needs a global approach taking into account, not only usual anthropometrical measurements allowing to define severity and the type of obesity (BMI and waist circumference) but also others parameters to evaluate the patient's morbid conscience and his level of motivation towards a potential treatment. Obese patient's possible therapeutic course is developed in this article according to three important parameters: eating disorders, motivation and capacity of introspection. The aim is to give tools to the generalist to select patients needing a multidisciplinary follow up in a specialized centre and those who can be treated in the office. PMID:16646369

  8. Contraception in the adolescent patient.

    PubMed

    Tafelski, T; Boehm, K E

    1995-03-01

    Having presented an overview of the available methods of contraception, the authors present one approach to prescribing contraceptives based on their experience. First, adolescent patients in our practice are discouraged from engaging in sexual intercourse. Abstinence is the only fail-safe method of contraception and provides benefits both emotionally and physically (i.e., prevention from sexually transmitted diseases and unwanted pregnancies). If the adolescent plans to be sexually active, however, she is encouraged to select some form of hormonal contraception, namely Norplant, Depo-Provera, or oral contraceptives, in conjunction with condoms. The authors have had little success recommending barrier methods alone. Condoms are used sporadically and the diaphragm is very awkward for adolescents who are still uncomfortable with their bodies and with touching themselves. Of the three hormonal methods, Depo-Provera seems to be well-accepted by our patients. It offers several advantages that we believe make it attractive. First, it does not require any forethought by the patient other than coming in for the injection every 3 months. There is an effective grace period, so the patient is afforded good contraception even if she is up to a month late for her injection. The frequent visits for injections actually can be looked upon as positive, frequent contacts with the patient and can provide opportunities for counseling. In addition, some patients become amenorrheic, which teens view as an advantage, increasing compliance. If it is not likely that a patient will be compliant with every-3-month injections, Norplant is recommended. Studies have shown good acceptance of Norplant by adolescents and that has been the experience of the authors. With appropriate counseling prior to insertion and a counseling session with patients who request removal, the number of actual Norplant removals has been limited. If Depo-Provera and Norplant are not acceptable to the patient, then oral contraceptives are recommended. Of the three methods, more opportunities for failure exist with oral contraceptives. Pills are missed for a variety of reasons, including going away for the weekend; not having a regular schedule, which can interrupt pill-taking; and even ambivalence about becoming pregnant. One recent study demonstrated certain patient characteristics that were associated with good compliance with oral contraceptives, including white race, higher education level, suburban residence, and older age of both the patient and her mate. Keeping these characteristics in mind may be helpful when prescribing oral contraceptives. Of course, it is the patient's prerogative to choose the type of contraception she feels will be best suited for her.(ABSTRACT TRUNCATED AT 400 WORDS) PMID:7777635

  9. [Psychotherapeutic treatment of borderline patients].

    PubMed

    Kernberg, O F

    1995-01-01

    The author gives in this paper an overview of current psychodynamic psychotherapy of patients with Borderline personality organization. Parallel to his own concept different treatment strategies by Adler, Volkan and Searles within the psychoanalytic framework are discussed. Finally recent developments of his own group are described. Emphasis lies on structuring the psychodynamic psychotherapeutic process of Borderline patients according to the developmental level the patient presents in each session and to treat symptoms belonging to so-called earlier levels (e.g primitive aggression, psychotic or paranoid transferences) before working through of more advanced conflicts. PMID:7761569

  10. Erectile dysfunction in COPD patients.

    PubMed

    Turan, Onur; Ure, Iyimser; Turan, Pakize Ayse

    2016-02-01

    Sexual dysfunction is a common problem in chronic obstructive pulmonary disease (COPD). We aimed to assess the presence of erectile dysfunction (ED) in COPD patients. Ninety-three outpatients who had been diagnosed as COPD and followed in Bolvadin State Hospital, Afyon, Turkey, were included in the study. All patients underwent pulmonary function tests and arterial blood gas analysis. They completed International Physical Activity Questionnaire (IPAQ), Medical Research Council (MRC) Dyspnea Scale, Short Form 36-item Scale (SF-36), and International Index of Erectile Function (IIEF) Questionnaire. The mean age of 10 (10.8%) mild, 46 (49.5%) moderate, 28 (30.1%) severe, and 9 (9.7%) very severe COPD patients was 61.4 ± 9.8 years. Varying degrees of ED were detected in 67.7% of COPD patients. All patients with hypoxemia had ED. IPAQ score and all SF-36 parameters were low in patients with ED, while MRC score was high. Forced expiratory volume in one second, forced vital capacity, partial pressure of oxygen in blood, oxygen (O2) saturation, IPAQ score, and role-physical parameters were statistically low in ED patients (p = 0.04, 0.02, <0.01, <0.01, 0.02, and 0.04, respectively); MRC score was statistically higher in patients with ED (p = 0.02). Patients with moderate and severe ED had statistically lower score of mental health (p < 0.01 and p = 0.02, respectively). There was a positive correlation between IIEF score and IPAQ scores (p < 0.01), MRC scores (p = 0.01), general health (p < 0.01), role-physical (p < 0.01), role-emotional (p < 0.01), physical functioning (p < 0.01), and mental health (p < 0.01) parameters in SF-36. ED is frequently seen in COPD patients. Hypoxemia, smoking, and limitation of physical activity are thought to be associated with ED in COPD as mechanisms. Quality of life and the functional capacity are negatively affected with the presence of ED. It is important for a physician to question the sexual functions in patients with COPD. The presence of ED may be routinely considered in the daily practice of pulmonologists in COPD patients. PMID:26647416

  11. With patient satisfaction under increasing scrutiny, consider patient callbacks.

    PubMed

    2011-07-01

    Under the final rules for Medicare's value-based purchasing program, one-third of the funding that is set aside to reward quality will be based on how patients rate their hospital experience. However, some EDs are already working to maximize patient satisfaction by implementing programs or policies whereby patients who have been discharged are routinely called to make sure their recovery is going well, as well as to intervene if there is an opportunity for service recovery. There are benefits to having ED clinicians make the follow-up calls themselves, but some health care organizations are also reaping benefits by having non-clinicians collect feedback on individual clinicians as well as specific facilities. In addition to potentially boosting quality and customer service, experts say callbacks are useful in curbing malpractice litigation. To avoid pushback among staff, consider beginning a program of patient callbacks by asking clinicians to call back just two patients per shift worked, and to share their experiences with colleagues. For maximum value, experts recommend that patient callbacks be made within one to four days of discharge. PMID:21749006

  12. Patient puzzle. Use systematic assessment to detect & correct patient conditions.

    PubMed

    Stoy, W A

    2001-01-01

    Medic 27 responds to a report of a fall victim at 27 West Pinnacle Lane. En route, the crew learns from dispatch that the patient fell approximately 25 feet from the roof of a three-story structure onto the roof of an adjacent garage. The caller reports the patient "going in and out of consciousness." The EMS crew requests the dispatch of a rescue unit and ladder company to assist on scene and the placement of a medical helicopter on standby. On scene, the patient's wife reports her husband accidentally disturbed a hornets' nest as he secured a weather vane to the top of the family home. She says the hornets stung her husband repeatedly. In his attempt to avoid the stings, his movements jarred the ladder, causing him to fall to the roof below. As you walk to the side of the patient's home, his wife adds that her husband has a cardiac condition and now complains of chest pain and trouble breathing. You wonder what you'll find when you reach the victim. Is he a medical patient with traumatic injuries or a trauma patient with medical complications? PMID:11213605

  13. Patient Navigation for Underserved Patients Diagnosed with Breast Cancer

    PubMed Central

    Raj, Aparna; Ko, Naomi; Battaglia, Tracy A.; Chabner, Bruce A.

    2012-01-01

    The elimination of cancer disparities is critically important for lessening the burden of breast cancer (BC). Patient navigator programs (PNPs) have been shown to improve rates of BC screening in underserved communities, but there is a dearth of evidence regarding their benefits after the actual diagnosis of BC. We retrospectively examined sociodemographic characteristics, disease characteristics, and concordance to quality measures (QMs) of BC care among women participating in a PNP that services disadvantaged minority communities in the greater Boston area. Of the 186 PNP patients diagnosed with BC in 20012011 in three neighborhood community health centers, treatment data was available for 158 (85%) and race and disease stage information was available for 149 (80%). Regarding stage, 25% were diagnosed with in situ cancer, 32% had stage 1, 25% had stage 2, 13% had stage 3, and 5% had stage 4 BC. Guideline-indicated care was received by 70 of 74 patients (95%) for the hormonal therapy QM, 15 of 17 (88%) patients for the chemotherapy QM, and 65 of 71 (92%) patients for the radiation QM, all similar to published concordance rates at elite National Comprehensive Cancer Network institutions. These findings suggest that PNPs may facilitate evidence-based quality care for vulnerable populations. Future research should prospectively analyze quality metrics to assess measures to improve the process and outcomes of patient navigation in diverse underserved settings, compared with control non-navigated populations. PMID:22752069

  14. Developing patient-centered teams: The role of sharing stories about patients and patient care.

    PubMed

    Bennett, Ariana H; Hassinger, Jane A; Martin, Lisa A; Harris, Lisa H; Gold, Marji

    2015-09-01

    Research indicates that health care teams are good for staff, patients, and organizations. The characteristics that make teams effective include shared objectives, mutual respect, clarity of roles, communication, trust, and collaboration. We were interested in examining how teams develop these positive characteristics. This paper explores the role of sharing stories about patients in developing patient-centered teams. Data for this paper came from 1 primary care clinic as part of a larger Providers Share Workshop study conducted by the University of Michigan. Each workshop included 5 facilitated group sessions in which staff met to talk about their work. This paper analyzes qualitative data from the workshops. Through an iterative process, research team members identified major themes, developed a coding scheme, and coded transcripts for qualitative data analysis. One of the most powerful ways group members connected was through sharing stories about their patients. Sharing clinical cases and stories helped participants bond around their shared mission of patient-centered care, build supportive relationships, enhance compassion for patients, communicate and resolve conflict, better understand workflows and job roles, develop trust, and increase morale. These attributes highlighted by participants correspond to those documented in the literature as important elements of teambuilding and key indicators of team effectiveness. The sharing of stories about patients seems to be a promising tool for positive team development in a primary care clinical setting and should be investigated further. PMID:26348238

  15. Connecting Patients With Specialty Products

    PubMed Central

    MCCAIN, JACK

    2012-01-01

    Previously, this series described distribution channels through which specialty drugs move to patients. This installment discusses changes and challenges that lie ahead, especially at the dispensing end. PMID:23091429

  16. Productivity and quality patient care.

    PubMed

    Pinette, Shirley L

    2003-01-01

    Because of the need to control rising U.S. health care costs, managers today not only must focus on their staff and patients, but also on the business aspects of radiology, such as increasing productivity. Balancing productivity with quality patient care is not an easy task--it requires changes by the entire radiology team, including managers, technologists and radiologists. After completing this article, the reader should be able to: Discuss why health care costs continue to rise. Define productivity, how it can be measured and why it must be measured in today's health care settings. Recognize how patient satisfaction contributes to a health care organization's bottom line. Understand the health care team's role in simultaneously increasing productivity and patient satisfaction. PMID:12800569

  17. Time out for patient safety.

    PubMed

    Meginniss, Anne; Damian, Frances; Falvo, Francine

    2012-01-01

    "Time out for patient safety" is a simple and effective tool to improve communication among caregivers based on the use of critical language that has been effective in the perioperative setting, airline industry, and military. In the emergency department it is non threatening and focuses attention on safe patient care. "Time out for patient safety" complements the use of other standardized communication techniques such as SBAR in clinical situations in which immediate intervention is mandatory for patient safety.This communication tool was presented to the multidisciplinary staff and has been embedded in the Emergency Department's simulation program for newly graduated nurses. The concept was well received by the group.Future research is needed to address outcomes for effectiveness. PMID:21764112

  18. Neurocognitive dysfunction in cancer patients.

    PubMed

    Meyers, C A

    2000-01-01

    Many cancer patients experience impairments of neurocognitive function, including memory loss, distractibility, difficulty in performing multiple tasks (multitasking), and a myriad of other symptoms. Patients may also concurrently suffer from mood disturbance and symptoms that compromise their ability to function adequately, including fatigue and pain. The etiologies of these problems are diverse and include the direct effects of cancer within the central nervous system (CNS), indirect effects of certain cancers (e.g., paraneoplastic brain disorders), and both diffuse and highly specific effects of cancer treatments on the brain. In addition to these cancer-related causes, patients may have coexisting neurologic or psychiatric disorders that affect their cognition and mood. Careful assessment of patients complaining of neurocognitive or behavioral problems is essential to providing appropriate interventions and maximizing their ability to carry out usual activities. PMID:10680150

  19. Doctor's Empathy Boosts Patient Satisfaction

    MedlinePlus

    ... they have staffers who excel in communication and customer service, he added. Ring also noted that health care reimbursement is increasingly linked to patient satisfaction and outcomes. SOURCE: American Academy of Orthopaedic Surgeons, ...

  20. Transfusion medicine in trauma patients

    PubMed Central

    Murthi, Sarah B; Dutton, Richard P; Edelman, Bennett B; Scalea, Thomas M; Hess, John R

    2011-01-01

    Injured patients stress the transfusion service with frequent demands for uncrossmatched red cells and plasma, occasional requirements for large amounts of blood products and the need for new and better blood products. Transfusion services stress trauma centers with demands for strict accountability for individual blood component units and adherence to indications in a clinical field where research has been difficult, and guidance opinion-based. New data suggest that the most severely injured patients arrive at the trauma center already coagulopathic and that these patients benefit from prompt, specific, corrective treatment. This research is clarifying trauma system requirements for new blood products and blood-product usage patterns, but the inability to obtain informed consent from severely injured patients remains an obstacle to further research. PMID:21083009

  1. Engaging patients through your website.

    PubMed

    Snyder, Kimberlee; Ornes, Lynne L; Paulson, Pat

    2014-01-01

    Legislation requires the healthcare industry to directly engage patients through technology. This paper proposes a model that can be used to review hospital websites for features that engage patients in their healthcare. The model describes four levels of patient engagement in website design. The sample consisted of 130 hospital websites from hospitals listed on 2010 and 2011 Most Wired Hospitals. Hospital websites were analyzed for features that encouraged patient interaction with their healthcare according to the levels in the model. Of the four levels identified in the model, websites ranged from "informing" to "collaborative" in website design. There was great variation of features offered on hospital websites with few being engaging and interactive. PMID:22913874

  2. The almost untreatable narcissistic patient.

    PubMed

    Kernberg, Otto F

    2007-01-01

    Clinical experience in the Personality Disorders Institute at Weill Cornell Medical College suggests that patients with borderline personality organization and a narcissistic personality disorder have a more serious prognosis than all other personality disorders functioning at the borderline level, and that those who in addition present significant antisocial behavior have an even worse prognosis (Clarkin, Yeomans, and Kernberg 1999; Stone 1990). This negative trend culminates in a group of practically untreatable patients with antisocial personality disorder, who represent the most severe cases of pathological narcissism. There are also patients with severe narcissistic personality disorder, functioning at an overt borderline level with significant antisocial features, but not presenting an antisocial personality disorder proper, who at times respond to treatment, while others do not. These patients are explored here, with a focus on particular psychotherapeutic techniques that have proven helpful, as well as on the limits of these technical approaches. PMID:17601104

  3. Polypharmacy in heart failure patients.

    PubMed

    Mastromarino, Vittoria; Casenghi, Matteo; Testa, Marco; Gabriele, Erica; Coluccia, Roberta; Rubattu, Speranza; Volpe, Massimo

    2014-06-01

    In heart failure (HF), the progressive use of multiple drugs and a complex therapeutic regimen is common and is recommended by international guidelines. With HF being a common disease in the elderly, patients often have numerous comorbidities that require additional specific treatment, thus producing a heavy pill burden. Polypharmacy, defined as the chronic use of five or more medications, is an underestimated problem in the management of HF patients. However, polypharmacy has an important impact on HF treatment, as it often leads to inappropriate drug prescription, poor adherence to pharmacological therapies, drug-drug interactions, and adverse effects. The growing complexity of HF patients, whose mean age increases progressively and who present multiple comorbidities, suggests the need for newer models of primary care to improve the management of HF patients. Self-care, telemonitoring, and natriuretic peptide-guided therapy represent promising new HF care models to face the complexity of the disease and its therapeutic regimen. PMID:24493574

  4. Bathing a patient in bed

    MedlinePlus

    Bed bath; Sponge bath ... beds to bathe. For these people, daily bed baths can help keep their skin healthy, control odor, ... pain, plan to give the patient a bed bath after the person has received pain medicine and ...

  5. Representing the patient in data

    NASA Astrophysics Data System (ADS)

    Hoffman, Kenneth J.

    1996-02-01

    In today's world, medical information on patients is usually recorded by a variety of health care providers who write their thoughts either on paper or in a word processing program. Telemedicine and other high technology initiatives add to the existing information database. However, most stored data are not easily linked. This makes it difficult to do process evaluations and nearly impossible to determine treatment effectiveness. Any aggregate data analysis must rely on the very few standardized patient data points that may include some demographic information, diagnosis and codable procedures. This paper demonstrates the utility of business process reengineering techniques using Integrated Computer Assisted Manufacturing Definition modeling to create a data model in which patient information is used to build an analyzable database. This provides the infrastructure in which to store all patient data.

  6. Paraneoplastic disorders in thymoma patients

    PubMed Central

    Lancaster, Eric; Evoli, Amelia

    2014-01-01

    Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis, acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor (AChR) for myasthenia gravis and to Caspr2, protein of the voltage-gated potassium channel (VGKC) complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to properly care for these patients. PMID:25396312

  7. Helping Patients To Quit Smoking

    PubMed Central

    Bass, Frederic

    1989-01-01

    Years ago, the tobacco leaf offered its users many social and ceremonial benefits. But today, throughout the world, the manufactured cigarette wreaks biological havoc. Clinically, physicians can make a small, but significant, contribution to their patients' stopping smoking. Family physicians who want to offer systematic aid to their smoking patients should assess the amount of time and energy they are willing to invest in patients' smoking and the probable rewards of such efforts. Behavioural change comprises four stages: a pre-motivational phase, a motivational phase, a behavioural-change phase, and a maintenance phase. Anyone who has ever smoked belongs to one of these phases and should be treated accordingly. Paradoxically, the physician should support consonant smoking (the patient freely choosing to smoke) except when the smoker is actively engaged in changing behaviour. PMID:21248907

  8. Corneal temperature in schizophrenia patients.

    PubMed

    Shiloh, Roni; Munitz, Hanan; Portuguese, Shirley; Gross-Isseroff, Ruth; Sigler, Mayanit; Bodinger, Liron; Katz, Nachum; Stryjer, Rafael; Hermesh, Haggai; Weizman, Abraham

    2005-12-01

    Most data imply that dopaminergic transmission is essential for proper hypothalamic-mediated core temperature regulation. Altered central dopaminergic transmission is suggested to be involved in the pathophysiology of schizophrenia. Thus, hypothetically, schizophrenia patients might be at increased risk of developing thermoregulatory dysregulation manifested by alterations in core temperature, as well as in peripheral tissue, the temperature of which has been shown to correlate with core temperature (e.g. cornea). Previous small pilot studies of ours showed that schizophrenia patients may exhibit corneal temperature abnormalities. Hence, we assessed corneal temperature in a controlled sample of drug-free ( n =11) and medicated ( n =28) schizophrenia patients compared to healthy comparison subjects ( n =9), using a FLIR thermal imaging camera. Drug-free schizophrenia patients exhibited significantly higher corneal temperature compared to healthy subjects, typical antipsychotic drug (APD)-treated patients ( n =16) and atypical APD-treated patients ( n =12) (37.08+/-1.46 degrees C vs. 33.37+/-2.51 degrees C, 31.08+/-1.43 degrees C and 31.67+/-0.44 degrees C respectively, p <0.0001; p <0.001 vs. each group separately). The healthy comparison subjects and the atypical APD-treated patients exhibited comparable corneal temperatures and these two groups exhibited higher corneal temperatures compared to the typical APD-treated patients ( p <0.01 and p =0.051 respectively). In conclusion, this study indicates that drug-free schizophrenia patients exhibit substantially higher corneal temperature compared to healthy comparison subjects or medicated patients, and that APDs may decrease corneal temperature either to normal (atypical APD) or to subnormal (typical APD) values. The relevance of these phenomena to the pathophysiology of schizophrenia, the biological mechanism underlying drug-induced corneal temperature alterations, the possible role of temperature-lowering drugs (neuroleptics or non-neuroleptics) on schizophrenic psychosis as well as the role of corneal temperature as a tool to evaluate adherence to APD treatment merit further investigation via larger samples of both medicated and drug-free schizophrenia patients compared to matched controlled subjects. PMID:15927092

  9. Information technologies and patient safety.

    PubMed

    Ellner, Scott J; Joyner, Paul W

    2012-02-01

    Advances in health information technology provide significant opportunities for improvements in surgical patient safety. The adoption and use of electronic health records can enhance communication along the surgical spectrum of care. Bar coding and radiofrequency identification technology are strategies to prevent retained surgical sponges and for tracking the operating room supply chain. Computerized intraoperative monitoring systems can improve the performance of the operating room team. Automated data registries collect patient information to be analyzed and used for surgical quality improvement. PMID:22269262

  10. Intragastric Balloon for Overweight Patients

    PubMed Central

    Martins Fernandes, Flavio Augusto; Lima, Diego L.; Rao, Prashanth; Shadduck, Phillip P.; Montandon, Isabelle D.; de Souza Barros, Juscielle; Rodrigues, Ingrid Lais Vieira

    2016-01-01

    Background and Objectives: Current treatments for overweight adults include reduced-calorie diet, exercise, behavior modification, and selective use of medications. Many achieve suboptimal results with these measures and progress to obesity. Whether the intragastric balloon (IGB), a reversible device approved for treatment of obesity, is a safe and effective option in overweight adults is less well studied. We conducted a study to prospectively analyze the safety and effectiveness of IGB in overweight adults, to compare the results to a simultaneously studied cohort of obese patients, and to share procedural tips for safe IGB placement and removal. Methods: One hundred thirty-nine patients were evaluated in this prospective, nonrandomized study. Twenty-six overweight [body mass index (BMI), 26–30)] and 113 obese (BMI > 30) patients underwent outpatient, endoscopic IGB placement under intravenous sedation. The IGB was filled with a 550–900 mL (average, 640 mL) solution of saline, radiological contrast, and methylene blue, with an approximate final proportion of 65:2:1. The patients were followed up at 1–2 weeks and then monthly for 6 months. At 6 months, they underwent IGB removal via an esophageal overtube to optimize safety, and then they were observed for 6 more months. Results: IGB time was 190 ± 36 d in the overweight patients and 192 ± 43 d in the obese patients. Symptoms of IGB intolerance included nausea and pain, which were transiently present in 50–95% of patients for several days, and necessitated early IGB removal in 6% of patients. There were no procedure-related complications and no IGB-related esophagitis, erosion, perforation, or obstruction. The percentage of excess weight loss (EWL%) was 96 ± 54% in the overweight group and 41 ± 26% in the obese group (P < 0.001). Conclusion: In overweight adults failing standard treatments, IGB placement for 6 months had an acceptable safety profile and excellent weight loss. PMID:26955259

  11. Colorectal Surgery in Cirrhotic Patients

    PubMed Central

    Steinhagen, Randolph M.

    2014-01-01

    Patients with cirrhosis have a greater risk of morbidity and mortality following colorectal surgery. Therefore, preoperative medical optimization and risk assessment using criteria such as the MELD score are vital in preventing complications. Some risk factors include age, urgency of surgery, and ASA score. Postoperative morbidity and mortality are related to portal hypertension, ascites, infection, and anastomotic and stomal complications. This review highlights the assessment of risk and perioperative management of cirrhotic patients undergoing colorectal surgery. PMID:24550693

  12. Pharmacometrics: Focus on the Patient

    PubMed Central

    Dumitrescu, T Pene; Fossler, MJ; Schmith, VD

    2015-01-01

    Pharmacometrics, whether using simple or complex models, has contributed to rational and efficient drug development,1–3 with the main focus on early drug development.4 This article describes why opportunities more directly focused on the patient abound in late stage development, illustrating the concept with three innovative examples which focus on benefits to patients, enabling drugs that are truly efficacious to reach the market faster in diseases with high unmet medical needs, while maintaining adequate safety. PMID:26225220

  13. [Anal cancer in HIV patients].

    PubMed

    Quéro, Laurent; Duval, Xavier; Abramowitz, Laurent

    2014-11-01

    Despite effective highly active antiretroviral treatment, anal cancer incidence has recently strongly increased in HIV-infected population. Treatment strategy in HIV-infected patients does not differ from general population. HIV-infected patients treated by chemo-radiotherapy are exposed to high-grade toxicities and should be closely monitored to deliver the optimal treatment. Close collaboration between oncologist and infectiologist is highly recommended to adjust antiretroviral therapy if necessary. PMID:25418596

  14. Patient Simulators Train Emergency Caregivers

    NASA Technical Reports Server (NTRS)

    2014-01-01

    Johnson Space Center teamed up with Sarasota, Florida-based METI (now CAE Healthcare) through the STTR program to ruggedize the company’s patient simulators for training astronauts in microgravity environments. The design modifications were implemented in future patient simulators that are now used to train first responders in the US military as well as fire departments and other agencies that work in disaster zones.

  15. Adenomyosis: What the Patient Needs.

    PubMed

    Alabiso, Giulia; Alio, Luigi; Arena, Saverio; Barbasetti di Prun, Allegra; Bergamini, Valentino; Berlanda, Nicola; Busacca, Mauro; Candiani, Massimo; Centini, Gabriele; Di Cello, Annalisa; Exacoustos, Caterina; Fedele, Luigi; Fuggetta, Eliana; Gabbi, Laura; Geraci, Elisa; Imperiale, Ludovica; Lavarini, Elena; Incandela, Domenico; Lazzeri, Lucia; Luisi, Stefano; Maiorana, Antonio; Maneschi, Francesco; Mannini, Luca; Mattei, Alberto; Muzii, Ludovico; Pagliardini, Luca; Perandini, Alessio; Perelli, Federica; Pinzauti, Serena; Porpora, Maria Grazia; Remorgida, Valentino; Leone Roberti Maggiore, Umberto; Seracchioli, Renato; Solima, Eugenio; Somigliana, Edgardo; Tosti, Claudia; Venturella, Roberta; Vercellini, Paolo; Viganò, Paola; Vignali, Michele; Zannoni, Letizia; Zullo, Fulvio; Zupi, Errico

    2016-01-01

    A panel of experts in the field of endometriosis expressed their opinions on management options in a 28-year-old patient, attempting pregnancy for 1 year, with severe cyclic pelvic pain and with clinical examination and imaging techniques suggestive of adenomyosis. Many questions this paradigmatic patient may pose to the clinician are addressed, and all clinical scenarios are discussed. A decision algorithm derived from this discussion is also proposed. PMID:26772777

  16. Neurophysiological approach to tinnitus patients.

    PubMed

    Jastreboff, P J; Gray, W C; Gold, S L

    1996-03-01

    The principal postulate of the neurophysiological model of tinnitus is that all levels of the auditory pathways and several nonauditory systems play essential roles in each case of tinnitus, stressing the dominance of nonauditory systems in determining the level of tinnitus annoyance. Thus it has been proposed to treat tinnitus by inducing and facilitating habituation to the tinnitus signal. The goal is to reach the stage at which, although patients may perceive tinnitus as unchanged when they focus on it, they are otherwise not aware of tinnitus. Furthermore, even when perceived, tinnitus does not evoke annoyance. Habituation is achieved by directive counseling combined with low-level, broad-band noise generated by wearable generators, and environmental sounds, according to a specific protocol. For habituation to occur, it is imperative to avoid masking tinnitus by these sounds. Since 1991, > 500 tinnitus patients have been seen in our center. About 40% exhibited hyperacusis to varying degrees. A survey of > 100 patients revealed > 80% of significant improvement in groups of patients treated with the full protocol involving counseling and the use of noise generators. Notably, in patients who received counseling only, the success rate was < 20%. The improvement in hyperacusis was observed in approximately 90% of treated patients. PMID:8723954

  17. Counseling patients about sexual issues.

    PubMed

    Stimmel, Glen L; Gutierrez, Mary A

    2006-11-01

    In recent years, the frequency of antidepressant drug-induced sexual dysfunction has increased, along with the use of new drugs for the treatment of erectile dysfunction and premature ejaculation. It has thus become common for pharmacists to counsel patients about sexual issues. Pharmacists must not only become knowledgeable about these drugs and their indications, but they must also become skilled and comfortable with counseling patients and answering questions from both patients and other health care providers. In addition to therapeutic information, pharmacists' discussions with patients should take into account factors that contribute to treatment nonadherence and treatment failure. Patient education is essential to ensure optimum outcomes for pharmacologic treatments for both erectile dysfunction and premature ejaculation. Improper use of phosphodiesterase-5 inhibitor drugs for erectile dysfunction accounts for most nonresponsiveness and discontinuation of treatment. Drug-induced sexual dysfunction is common with some psychotropic drugs. Up to 50% of men will experience delayed ejaculation, and at least 30% of men and women will experience anorgasmia from antidepressant drugs with serotonin agonist activity. Trazodone is the drug most commonly associated with the rare but very serious adverse effect of priapism. The pharmacist who is both competent and comfortable discussing sexual function and dysfunction with patients can make positive contributions to their therapeutic outcomes as well as their quality of life. PMID:17064206

  18. Towards patient safety in anaesthesia.

    PubMed

    Cooper, J B

    1994-07-01

    The anaesthesia specialty has focused on the safety of the patient and examination of untoward outcomes. Serious injuries are now rare in medically advanced countries. Still, anaesthesia deaths and complications are important because the anaesthetic itself has no intended therapeutic effect. Safety is a never-ending battle that requires continued effort because many forces have the potential to diminish whatever progress is made. This paper describes the modern movement in anaesthesia patient safety--the reasons it started, the major foci and explanations for why anaesthesia seems now to be safer than at any time in history. The American legal system, critical incident studies, studies of malpractice claims and large-scale studies of anaesthesia outcomes played a role in increasing the awareness of the need to enhance anaesthesia safety. Many efforts are believed to have contributed to improvements in the safety of anaesthesia: improved training of anaesthesia clinicians, new pharmaceuticals, new technologies for monitoring (especially pulse oximetry and capnography), standards for monitoring and other aspects of anaesthesia care, safety enhancements in anaesthesia equipment and the implementation of quality assurance and risk management programmes. The creation of the Anesthesia Patient Safety Foundation in the United States and a similar organization in Australia have helped to bring about awareness of safety issues and to support study of patient safety. Ultimately, the motto of the Anesthesia Patient Safety Foundation should be the goal of all anaesthesia professionals: "That no patient shall be harmed by anaesthesia". PMID:7979131

  19. Peripapillary Retinoschisis in Glaucoma Patients

    PubMed Central

    Bayraktar, Serife; Cebeci, Zafer; Kabaalioglu, Melis; Ciloglu, Serife; Kir, Nur; Izgi, Belgin

    2016-01-01

    Purpose. To investigate peripapillary retinoschisis and its effect on retinal nerve fiber layer (RNFL) thickness measurements by using spectral-domain optical coherence tomography (SD-OCT) in glaucomatous eyes. Methods. Circumpapillary RNFL (cpRNFL) B-scan images of 940 glaucoma patients (Group 1) and 801 glaucoma-suspect patients (Group 2) obtained by SD-OCT were reviewed. The structural and clinical characteristics of the retinoschisis were investigated. The RNFL thickness measurements taken at the time of retinoschisis diagnosis and at the follow-up visits were also compared. Results. Twenty-nine retinoschisis areas were found in 26 of the 940 glaucoma patients (3.1%) in Group 1 and seven areas were found in 801 patients (0.87%) in Group 2. In glaucomatous eyes, the retinoschisis was attached to the optic disc and overlapped with the RNFL defect. At the time of retinoschisis, the RNFL thickness was statistically greater in the inferior temporal quadrant when compared with the follow-up scans (p < 0.001). No macular involvement or retinal detachment was observed. Conclusion. The present study investigated 33 peripapillary retinoschisis patients. Increase in RNFL thickness measurements was observed at the time of retinoschisis. It is important to examine the cpRNFL B-scan images of glaucoma patients so that the RNFL thickness is not overestimated. PMID:27069674

  20. Establishing an active patient partnership.

    PubMed

    Herrier, R N; Boyce, R W

    1995-04-01

    Pharmacists face many changes in the coming decade, some of which threaten their professional survival. Although uncertainty may currently prevail, one of these changes, the shift in the patient-health care professional relationship from the patient taking a passive role to an active partnering role, provides pharmacists with many opportunities to realize the vision of patient-centered care that has been advocated by pharmacy innovators and leaders for almost three decades. To take advantage of these changes, pharmacists must modify their practice paradigms and use their existing strengths, such as easy patient access and high levels of patient trust, to help develop a new model of pharmaceutical care. The concern that the magnitude of these changes will prevent successful practice transformations may be exaggerated. In reality, these proposed "new" roles have been in existence for much of this century. Most pharmacists can expand and enhance their traditional roles as self-care advisors and patient educators simply by incremental improvements in interpersonal and clinical skills. Rather than a Star Trek approach to "go where no man has gone before," the profession needs only a pharmaceutical sequel to Back to the Future. PMID:7754972

  1. [Fertility in testicular cancer patients].

    PubMed

    Shin, Takeshi; Miyata, Akane; Arai, Gaku; Okada, Hiroshi

    2015-03-01

    Testicular cancer(TC)is the most common and curable cancer affecting men of reproductive age. Successful treatment approaches have resulted in longer life expectancy in TC survivors. The most frequently used treatment for TC is a combination of inguinal orchiectomy, and either radiotherapy or cisplatin-based chemotherapy. In many TC patients, sperm quality is already abnormal and there may even be a lack of viable spermatozoa at the time of diagnosis. Therefore, the effect of cancer treatment on fertility is a potentially significant issue. Fertility preservation in these men has become essential and needs to be discussed prior to the start of cancer treatment. The only currently established fertility preservation method is the cryopreservation of sperm before therapy. For most patients seeking cryopreservation, the semen sample is collected via masturbation. If the patient is unable to ejaculate for any reason, other techniques such as vibratory stimulation and electroejaculation can be performed. In azoospermic or severely oligozoospermic patients, testicular sperm extraction at the time of the inguinal orchiectomy is a useful technique for obtaining spermatozoa before cytotoxic therapy. We herein present an overview of the current topics on fertility in TC patients, including the effects of surgery, chemotherapy, and radiation therapy. We also describe the strategy for fertility preservation in these patients. PMID:25812494

  2. Optimising patient outcomes in myeloma.

    PubMed

    Harousseau, Jean-Luc

    2010-05-01

    Multiple myeloma (MM) is an incurable disease, and the goal of therapy is to prolong survival. Newer therapies (thalidomide, lenalidomide, and bortezomib) have contributed to the recent improvements in survival. Optimal integration of these newer therapies into standard practice may be aided by better methods of risk stratification. Supplementation of existing risk stratification methods with new prognostic information, such as cytogenetic data and gene expression profiles, may improve prognostication and help to identify appropriate treatment. The advent of newer therapies has also prompted a reassessment of traditional endpoints and goals of therapy, such as complete response. While complete response correlates with survival in some cases, the correlation is not consistent across all treatment regimens and patient groups, and is therefore not always the most appropriate goal of therapy. With the aim of prolonging survival, trials are currently evaluating newer therapies as long-term maintenance therapy or as prevention therapy for patients with smouldering myeloma. Given that these patients are often asymptomatic and free of clinically active disease, success in this setting depends highly on long-term tolerability of these agents. The available evidence suggests that their adverse event profiles are distinct, predictable, and manageable with careful monitoring and intervention as appropriate. Treatment of MM should therefore be tailored to the individual patient based on the goals of therapy, patient condition, expected adverse events, and patient preference. PMID:20472187

  3. Spiritual care in hospitalized patients

    PubMed Central

    Yousefi, Hojjatollah; Abedi, Heidar Ali

    2011-01-01

    BACKGROUND: Spiritual needs are among an individual's essential needs in all places and times. With his physical and spiritual dimensions and the mutual effect of these two dimensions, human has spiritual needs as well. These needs are an intrinsic need throughout the life; therefore, they will remain as a major element of holistic nursing care. One of the greatest challenges for nurses is to satisfy the patients’ spiritual needs. METHODS: This is a qualitative study with hermeneutic phenomenological approach. Data were collected from 16 patients hospitalized in internal medicine-surgery wards and 6 nurses in the respective wards. Data were generated by open-ended interview and analyzed using Diekelmann's seven-stage method. Rigorousness of findings was confirmed by use of this method as well as team interpretation, and referring to the text and participants. RESULTS: In final interpretation of the findings, totally 10 sub-themes, three themes including formation of mutual relation with patient, encouraging the patient, and providing the necessary conditions for patient's connection with God, and one constitutive pattern, namely spiritual need of hospitalized patients. CONCLUSIONS: Spiritual needs are those needs whose satisfaction causes the person's spiritual growth and make the person a social, hopeful individual who always thanks God. They include the need for communication with others, communication with God, and being hopeful. In this study, the three obtained themes are the spiritual needs whose satisfaction is possible in nursing system. Considering these spiritual aspects accelerates patient's treatment. PMID:22039390

  4. [Pain Management in geriatric patients].

    PubMed

    Eiche, Jürgen; Schache, Florian

    2016-04-01

    Pains belong to the most frequent reasons for a doctor's visit. In elderly people, it is the result of progressive degenerative processes (e. g. , arthrosis, Osteoarthritis, degenerative spinal changes) and a higher prevalence of cancer disease to a further increase of the patients who suffer unnecessarily from pains. By the increasing polymorbidity (e.g. diabetes mellitus, vascular disease) and a declining immune competence, the prevalence of polyneuropathy and post-herpetic neuralgia rises. Insufficiently treated chronic or periodically returning pain can lead to serious interferences of the physical, cognitive and social everyday competence and therefore to a limited quality of life. These facts shows the relevance of a sufficient pain therapy in geriatric patients. Nevertheless, on account of existing comorbidity, polypharmacy as well as of impaired organ function, the pharmacological pain therapy in old patients also poses a potential hazard. Although pain prevalence is higher with geriatric than with younger patients, significantly less analgesics are prescribed in the elderly population. This results from existing uncertainties at the treating doctors as well as the complicated pain capture, in particular with cognitive affected patients. The present article should indicate options of treatment for geriatric pain patients. PMID:27123730

  5. [Delusion in the critical patient].

    PubMed

    Palencia-Herrejón, E; Romera, M A; Silva, J A

    2008-02-01

    Delirium, the acute confusional syndrome, is a common although infradiagnosed problem in the critically ill patient, especially the hypoactive subtype. Risk factors for delirium are previous cognitive disturbances, some comorbidities, ambiental factors and the acute organic alterations of critical illness. Delirium is associated to an increase in short and long term mortality, prolongation of mechanical ventilation, increased Intensive Care Unit (ICU) and hospital length of stay, and cognitive impairment after hospital discharge. In the last years several tools have been developed to detect delirium in critically ill patients. The Intensive Care Delirium Screening Checklist (ICDSC) and the Confusion Assessment Method for ICU patients (CAM-ICU) have been validated and are useful even in patients receiving mechanical ventilation. Some interventions on specific risk factors can decrease the incidence of delirium in hospitalized patients. Treatment of delirium is based on the identification and correction of contributing factors, the introduction of support measures, and pharmacological therapy for symptomatic control. Halloperidol is the first line therapy of delirium in the critically ill patient, while experience with atypical neuroleptics and other drugs is limited, precluding to do recommendations about its use. Neuroleptic drugs can produce severe side effects and need careful dosage and monitoring. When agitation is important, can be necessary the simultaneous use of benzodiazepines or propofol, and some times, the temporal and protocolized application of physical restraints. PMID:18405541

  6. Hypothermia and the trauma patient

    PubMed Central

    Kirkpatrick, Andrew W.; Chun, Rosaleen; Brown, Ross; Simons, Richard K.

    Hypothermia has profound effects on every system in the body, causing an overall slowing of enzymatic reactions and reduced metabolic requirements. Hypothermic, acutely injured patients with multisystem trauma have adverse outcomes when compared with normothermic control patients. Trauma patients are inherently predisposed to hypothermia from a variety of intrinsic and iatrogenic causes. Coagulation and cardiac sequelae are the most pertinent physiological concerns. Hypothermia and coagulopathy often mandate a simplified approach to complex surgical problems. A modification of traditional classification systems of hypothermia, applicable to trauma patients is suggested. There are few controlled investigations, but clinical opinion strongly supports the active prevention of hypothermia in the acutely traumatized patient. Preventive measures are simple and inexpensive, but the active reversal of hypothermia is much more complicated, often invasive and controversial. The ideal method of rewarming is unclear but must be individualized to the patient and is institution specific. An algorithm reflecting newer approaches to traumatic injury and technical advances in equipment and techniques is suggested. Conversely, hypothermia has selected clinical benefits when appropriately used in cases of trauma. Severe hypothermia has allowed remarkable survivals in the course of accidental circulatory arrest. The selective application of mild hypothermia in severe traumatic brain injury is an area with promise. Deliberate circulatory arrest with hypothermic cerebral protection has also been used for seemingly unrepairable injuries and is the focus of ongoing research. PMID:10526517

  7. Difficult physician-patient relationships.

    PubMed

    Reifsteck, S W

    1998-01-01

    Changes in the delivery of health care services in the United States are proceeding so rapidly that many providers are asking how the working relationships between doctors and patients will be effected. Accelerated by cost containment, quality improvement and the growth of managed care, these changes have caused some critics to feel that shorter visits and gatekeeper systems will promote an adversarial relationship between physicians and patients. However, proponents of the changing system feel that better prevention, follow-up care and the attention to customer service these plans can offer will lead to increased patient satisfaction and improved doctor-patient communication. Dedicated to addressing these concerns, the Bayer Institute for Health Care Communication was established in 1987 as a continuing medical education program (CME) focusing on this topic. A half-day workshop on clinician-patient communication to enhance health outcomes was introduced in 1992 and a second workshop, "Difficult' Clinician-Patient Relationships," was developed two years later. The two courses discussed in this article are offered to all physicians, residents, medical students, mid-level providers and other interested staff within the Carle system. PMID:10186308

  8. Regional anesthesia for the trauma patient: improving patient outcomes.

    PubMed

    Gadsden, Jeff; Warlick, Alicia

    2015-01-01

    Trauma is a significant health problem and a leading cause of death in all age groups. Pain related to trauma is frequently severe, but is often undertreated in the trauma population. Opioids are widely used to treat pain in injured patients but have a broad range of undesirable effects in a multitrauma patient such as neurologic and respiratory impairment and delirium. In contrast, regional analgesia confers excellent site-specific pain relief that is free from major side effects, reduces opioid requirement in trauma patients, and is safe and easy to perform. Specific populations that have shown benefits (including morbidity and mortality advantages) with regional analgesic techniques include those with fractured ribs, femur and hip fractures, and patients undergoing digital replantation. Acute compartment syndrome is a potentially devastating sequela of soft-tissue injury that complicates high-energy injuries such as proximal tibia fractures. The use of regional anesthesia in patients at risk for compartment syndrome is controversial; although the data is sparse, there is no evidence that peripheral nerve blocks delay the diagnosis, and these techniques may in fact facilitate the recognition of pathologic breakthrough pain. The benefits of regional analgesia are likely most influential when it is initiated as early as possible, and the performance of nerve blocks both in the emergency room and in the field has been shown to provide quality pain relief with an excellent safety profile. PMID:26316813

  9. Patients Perception About Coronary Artery Bypass Grafting

    PubMed Central

    Mendona, Kelminda Maria Bulhes; de Andrade, Tarcisio Matos

    2015-01-01

    OBJECTIVE The diagnosis of coronary artery disease referred for heart surgery has an important psychological component. The purpose of this study was to access the difficulties experienced by individuals awaiting coronary artery bypass grafting and to determine strategies that facilitate adaptation to a new lifestyle, modified by the disease. METHODS A qualitative, exploratory study involving patients admitted to a university teaching hospital in the city of Salvador, Bahia, Brazil, awaiting coronary artery bypass grafting. Semi-structured interviews were performed in accordance with a previously defined script based on the study objective. Each transcription was read in its entirety to verify the representativeness, homogeneity and pertinence of the data obtained (pre-analysis), followed by separation of categories of analysis. RESULTS The descriptions of this study show that patients admitted to the completion of coronary artery bypass grafting experience a wide range of psychological difficulties, considering that surgery acquires interpretations that vary according to individuals' subjectivity. The patients recognized the benefit of being able to discuss their feelings as a means of diminishing their fear and anxiety. CONCLUSION Helping patients find resources to confront more positively the daily hospitalization is an important aspect for the health care professionals who assist them. This goal can be achieved through modification of the biomedical model of care for a biopsychosocial view. The investment of time and attention is of fundamental importance and aims to overcome existing deficiencies that interfere with the outcome of patients after cardiac surgery. PMID:26735601

  10. Wireless patient monitoring system for patients with nasal obstruction.

    PubMed

    Choi, Hyuk; Park, Il-Ho; Yoon, Hu Geun; Lee, Heung-Man

    2011-01-01

    A new system for nasal sound analysis via Internet to cellular phone was investigated. Spectral analysis of the nasal sound with frequency domain and dB is an important factor in the investigation of nasal airflow pattern. This study included 10 patients and 10 healthy subjects. Patients underwent nasal septoplasty surgery for treatment of nasal septal deviation. This nasal sound analysis was performed on subjects at 1 month postsurgery. This study was performed using an investigator-developed software that sends real-time frequency and spectral analysis video of a patient's nasal sound to an otolaryngologist's cellular phone. Sound intensity was observed at over 25 dB with high range of frequency (2-4 kHz) and less than 10 dB with low (500-1,000 Hz) and medium (1-2 kHz) frequency from 10 patients with nasal obstruction symptoms (group A). In 10 healthy subjects without nasal obstruction symptoms (group B), sound intensity was observed at high frequencies below 5 dB; however, low and medium frequencies were above 15 dB. A statistically significant difference in sound intensity was observed between group A and group B. It was ascertained that use of the new technique will help patients to avoid an unnecessary return to the hospital and will also save money and time. PMID:21214305

  11. Regional anesthesia for the trauma patient: improving patient outcomes

    PubMed Central

    Gadsden, Jeff; Warlick, Alicia

    2015-01-01

    Trauma is a significant health problem and a leading cause of death in all age groups. Pain related to trauma is frequently severe, but is often undertreated in the trauma population. Opioids are widely used to treat pain in injured patients but have a broad range of undesirable effects in a multitrauma patient such as neurologic and respiratory impairment and delirium. In contrast, regional analgesia confers excellent site-specific pain relief that is free from major side effects, reduces opioid requirement in trauma patients, and is safe and easy to perform. Specific populations that have shown benefits (including morbidity and mortality advantages) with regional analgesic techniques include those with fractured ribs, femur and hip fractures, and patients undergoing digital replantation. Acute compartment syndrome is a potentially devastating sequela of soft-tissue injury that complicates high-energy injuries such as proximal tibia fractures. The use of regional anesthesia in patients at risk for compartment syndrome is controversial; although the data is sparse, there is no evidence that peripheral nerve blocks delay the diagnosis, and these techniques may in fact facilitate the recognition of pathologic breakthrough pain. The benefits of regional analgesia are likely most influential when it is initiated as early as possible, and the performance of nerve blocks both in the emergency room and in the field has been shown to provide quality pain relief with an excellent safety profile. PMID:26316813

  12. Antimicrobial Dose in Obese Patient

    PubMed Central

    Kassab, Sawsan; Syed Sulaiman, Syed Azhar; Abdul Aziz, Noorizan

    2007-01-01

    Introduction Obesity is a chronic disease that has become one of major public health issue in Malaysia because of its association with other disease states including cardiovascular disease and diabetes. Despite continuous efforts to educate the public about the health risks associated with obesity, prevalence of the disease continues to increase. Dosing of many medications are based on weight, limited data are available on how antimicrobial agents should be dosed in obesity. The aim of this case presentation is to discuss dose of antibiotic in obese patient. Case report: Patient: GMN, Malay, Female, 45 year old, 150kg, transferred from medical ward to ICU with problems of fever, orthopnea, sepsis secondary to nosocomial pneumonia. She was admitted to hospital a week ago for SOB on exertion, cyanosis, mildly dyspneic, somasthenia, bilateral ankle swelling. There was no fever, cough, chest pain, clubbing, flapping tremor. Her grand father has pre-morbid history of obesity, HPT, DM and asthma. She was non alcoholic, smoker, and not on diet control. The diagnosis Pickwickian syndrome was made. Patient was treated with IV Dopamine 11mcg/kg/min, IV Morphine 4mg/h. IV GTN 15mcg/min, IV Ca gluconate 10g/24h for 3/7, IV Zantac 50mg tds, IV Augmentin 1.2g tds, IV Lasix 40mg od, IV Plasil 10mg tds, S.c heparin 5000IU bd. patient become stable and moved to medical ward to continue her treatment. Discussion: The altered physiologic function seen in obese patients is a concern in patients receiving antimicrobial agents because therapeutic outcomes depend on achieving a minimum inhibitory concentration (MIC). The therapeutic effect of any drug can be altered when any of the 4 pharmacokinetic processes (absorption, distribution, metabolism, or elimination) are altered. Decreased blood flow rates and increased renal clearance in obese patients can affect drug distribution and elimination. Changes in serum protein levels can change the metabolism and distribution of drugs that are highly protein bound; the effects of these physiologic differences should be considered when administrating antimicrobial agents in obese patients. Conclusion: Generally this patient was moderately well managed in view of inappropriate combination of antibiotic, duplication or unnecessary use of antibiotic for treatment of sepsis, more clinical studies are needed to determine antibiotic dose in obese patient.

  13. Postoperative analgesia in elderly patients.

    PubMed

    Falzone, Elisabeth; Hoffmann, Clément; Keita, Hawa

    2013-02-01

    Elderly people represent the fastest-growing segment of our society and undergo surgery more frequently than other age groups. Effective postoperative analgesia is essential in these patients because inadequate pain control after surgery is associated with adverse outcomes in elderly patients. However, management of postoperative pain in older patients may be complicated by a number of factors, including a higher risk of age- and disease-related changes in physiology and disease-drug and drug-drug interactions. Physiological changes related to aging need to be carefully considered because aging is individualized and progressive. Assessment of pain management needs to include chronological age, biological age with regard to renal, liver and cardiac functions, and the individual profile of pathology and prescribed medications. In addition, ways in which pain should be assessed, particularly in patients with cognitive impairment, must be considered. Cognitively intact older patients can use most commonly used unidimensional pain scales such as the visual analogue scale (VAS), verbal rating scale (VRS), numeric rating scale (NRS) and facial pain scale (FPS). VRS and NRS are the most appropriate pain scales for the elderly. In older patients with mild to moderate cognitive impairment, the VRS is a better tool. For severe cognitively impaired older patients, behavioural scales validated in the postoperative context, such as Doloplus-2 or Algoplus, are appropriate. For postoperative pain treatment, most drugs (e.g. paracetamol, nonsteroidal anti-inflammatory drugs, nefopam, tramadol, codeine, morphine, local anaesthetics), techniques (e.g. intravenous morphine titration, subcutaneous morphine, intravenous or epidural patient-controlled analgesia, intrathecal morphine, peripheral nerve block) and strategies (e.g. anticipated intraoperative analgesia or multimodal analgesia) used for acute pain management can be used in older patients. However, in view of pharmacokinetic and pharmacodynamic changes in older persons, the higher incidence of co-morbidities and concurrent use of other drugs, each must be carefully adjusted to suit each patient. Evaluation of treatment efficacy and incidence and severity of adverse events should be monitored closely, and the concept of 'start low and go slow' should be adopted for most analgesic strategies. PMID:23288604

  14. National Council on Patient Information and Education

    MedlinePlus

    ... to the National Council on Patient Information and Education One of the original patient safety coalitions, NCPIE ... 1999-2016 National Council on Patient Information and Education. All rights reserved. | Privacy Policy NCPIE 200-A ...

  15. Message for Heart Failure Patients: Exercise

    MedlinePlus

    ... gov/medlineplus/news/fullstory_159059.html Message for Heart Failure Patients: Exercise You'll feel better and maybe ... activity may help extend survival for patients with heart failure, a new review suggests. "Patients with heart failure ...

  16. Antinuclear antibodies in rosacea patients

    PubMed Central

    Salamon, Małgorzata; McCauliffe, Daniel; Sysa-Jędrzejowska, Anna

    2013-01-01

    Introduction Rosacea is a common inflammatory disorder, characterized by a spectrum of facial manifestations. The clinical similarity to other dermatoses, like lupus erythematosus, might lead to misdiagnosis, particularly in patients with elevated antinuclear antibody titers. Aim To assess the frequency, titer and specificity of antinuclear antibodies in rosacea patients and correlate these findings with clinical features. Material and methods The study included 101 rosacea patients and 26 sex- and age-matched controls. Immunofluorescence antinuclear antibody testing was performed on HEp-2 substrates. Patients’ sera with ANA titers of 1 : 160 or higher were evaluated by Euroline analysis. Results Over a half (53.5%) of rosacea patients had an ANA titer greater than or equal to 1 : 160. Within this group 13.86% had a titer of 1 : 320, 8.91% had a titer of 1 : 640, and 6.93% had a titer of 1 : 1,280 or higher. The specificity of these antibodies could not be identified. Elevated ANA titers were present more often in women (55.8%) than in men (44.15%). Only two of 26 healthy volunteers had elevated ANA titers. One had a titer of 1 : 160 and the other of 1 : 320. During a two-year observation period, after the initial ANA testing, none of the patients with ANA titers above 1 : 640 developed an apparent autoimmune disorder. Conclusions Elevated ANA titers are commonly found in rosacea patients, what with simultaneously existing facial erythema and photosensitivity might lead to misdiagnosis of lupus erythematosus. Clinicians should beware of these findings to avoid misdiagnosing lupus erythematosus in rosacea patients with elevated ANA titers. PMID:24278039

  17. Patient-Physician Web Messaging

    PubMed Central

    Liederman, Eric M; Lee, Jerry C; Baquero, Victor H; Seites, Paul G

    2005-01-01

    BACKGROUND Patients want electronic access to providers. Providers fear being overwhelmed by unreimbursed messages. OBJECTIVE Measure the effects of patient-physician web messaging on primary care practices. DESIGN/SETTING Retrospective analysis of 6 case and 9 control internal medicine (IM) and family practice (FP) physicians' message volume, and a survey of 5,971 patients' web messaging with 267 providers and staff in 16 community primary care clinics in the Sacramento, CA region. MEASUREMENTS AND MAIN RESULTS Case telephone volume was 18.2% lower (P =.002) and fell 6.50 times faster than control. Case total telephone plus web message volume was 13.7% lower (P =.025) and fell 5.84 times faster than control. Surveys were responded to by 40.3% (1,743/4,320) of patients and 61.4% (164/267) of providers and staff. Patients were overwhelmingly satisfied and providers and staff were generally satisfied; both found the system easy to use. Patient satisfaction correlated strongly with provider response time (Γ=0.557), and provider/staff satisfaction with computer skills (Γ=0.626) (Goodman-Kruskal Gamma [Γ] measure of ordinal association). CONCLUSIONS Secure web messaging improves on e-mail with encryption, access controls, message templates, customized message and prescription routing, knowledge content, and reimbursement. Further study is needed to determine whether reducing telephone traffic through the use of web messaging decreases provider interruptions and increases clinical efficiency during the workday. Satisfaction with web messaging may increase patient retention. PMID:15693928

  18. Osteodystrophy in Indonesian haemodialysis patients.

    PubMed

    Santoso, Djoko; Yogiantoro, Moh; Tomino, Yasuhiko

    2003-10-01

    A preliminary study of the intact-parathyroid hormone (i-PTH) measurements from haemodialysis patients was conducted to determine the prevalence of renal bone diseases at the Dr Soetomo Hospital. The objective of this study is to evaluate the osteodystrophy renal pattern in haemodialysis patients using i-PTH and radiological parameters. The selected populations of 48 (32 males and 16 females), the mean age 48 +/- 10.3 years, was evaluated to conduct a cross-sectional study. The calorimetric method was applied to measure serum P and Ca, while a radioimmunoassay was used to assay the i-PTH level. Of those 48 patients receiving haemodialysis, with a duration ranging from 4 to 432 weeks, 61% had hypocalcaemia and 10% had hypercalcaemia. The i-PTH levels below 100 pg/mL (normal, 10-65 pg/mL) suggested 'aplastic' bone, and values of 100-200 pg/mL most commonly indicated 'normal' bone turnover. The i-PTH levels over 200 pg/mL suggested hyperparathyroidism. The results of this study demonstrated that 42% of those patients had <100 pg/mL (low turnover bone presumed, no biopsy), 23% had 100 - <200 pg/mL ('normal' bone turnover) and 35% of them had >200 pg/mL ('hyperparathyroidism'). In addition, the radiological study showed that 10% of those patients were positive for renal bone diseases. In conclusion, this study shows that the common type of renal osteodystrophy was of a low turnover type, which was different from the findings in other previous studies. It is postulated that this difference is likely to be caused by some factors such as the general health condition of the population those patients belong to and, in particular, the nutritional status of those patients. PMID:15012715

  19. Heartsink – patient, doctor or consultation?

    PubMed

    Lee, Rachel

    2012-07-01

    I recently participated in a registrar education discussion about 'heartsink' patients. What is a heartsink patient? O'Dowd appears to have coined the phrase and refers to patients who 'exasperate, defeat and overwhelm their doctors by their behaviour'. He implemented a plan to identify, discuss and actively manage the heartsink patients at his practice and his definition has led to a classification of typical trigger patients and guidelines on how to best manage these patients PMID:22937552

  20. Patient Informatics: Technology in the Service of Patient Care

    PubMed Central

    Brennan, Patricia Flatley

    1990-01-01

    Care of the patient at home challenges the health care system with both the quantity and diversity of services required. Informatics technologies may provide mechanisms to relieve the burden of traditional services while meeting the unique needs of home-based patients in a timely and effective manner. Capitalizing on an existing, free, public-access computer network we developed the COMPUTERLINK, a set of utilities designed to provide home-care support to persons living with AIDS/ARC (PLWA) in the community. The pilot study presented here we demonstrate the feasibility of using home-based computer networks to provide information, communication and decision assistance to PLWA. The success experienced with this particular group provides sufficient encouragement to extend this intervention to other groups of community-based patients.

  1. Narcolepsy: Let the Patients Voice Awaken Us!

    PubMed Central

    Flygare, Julie; Parthasarathy, Sairam

    2014-01-01

    This is a patient-centered review about narcolepsy that aims to awaken the reader to the narcolepsy condition and to the trials and tribulations of patients with sleep problems in general. Narcolepsy is a neurological disorder with a classic tetrad of symptoms consisting of excessive daytime sleepiness, cataplexy, sleep onset hallucinations, and sleep paralysis. The diagnosis of narcolepsy and other sleep disorders are often overlooked and could be attributed to other medical or even psychiatric conditions with years of missed diagnosis. Implementation of two sleep-related questions to the review of systems in the primary care physicians office visit may help address the issue of missed diagnosis and allow patients to seek prompt medical attention. Definitive diagnosis can be made by overnight sleep study followed by a nap test, multiple sleep latency test (MSLT). There is currently no cure for narcolepsy with the treatments addressing symptoms of excessive daytime sleepiness, cataplexy, and nighttime sleep disruption with stimulants (modafinil, methylphenidate, and amphetamines), anti-cataplexy medications (Serotonin-specific reuptake inhibitors and tricyclic antidepressants) and sedative-hypnotics including sodium oxybate. Narcolepsy, like other sleep disorders, can lead to marked reductions of health-related quality of life and affect patients social and work lives deleteriously. While traditional healthcare approaches are focused more on hard biomedical outcomes, a patient-centered approach with novel methods for better sleep assessment of patients, that can bypass the impossibly crammed physician office visit, would allow healthcare providers to better detect, diagnose and treat narcolepsy and other such sleep problems. PMID:24931392

  2. Reducing patient suffering through compassionate connected care.

    PubMed

    Dempsey, Christina; Wojciechowski, Sharyl; McConville, Elizabeth; Drain, Maxwell

    2014-10-01

    Patient experience continues to play an increasingly critical role in quality outcomes and reimbursement. Nurse executives are tasked with helping direct-care nurses connect with patients to improve care experiences. Connecting with patients in compassionate ways to alleviate inherent patient suffering and prevent avoidable suffering is key to improving the patient experience. The Compassionate Connected Care framework identifies strategies for meeting the challenges of connecting with patients and reducing suffering. Methods integrate clinical, operational, cultural, and behavioral aspects of care to target patient needs based on condition. Caregivers learn to better express empathy and compassion to patients, and nurse leaders are better equipped to engage nurses at the bedside. PMID:25208270

  3. Nutritional Status in Cirrhotic Patients

    PubMed Central

    TEIUSANU, Adriana; ANDREI, Mihai; ARBANAS, Tudor; NICOLAIE, Tudor; DICULESCU, Mircea

    2012-01-01

    ABSTRACT Background: Malnutrition is prevalent in all forms of liver disease: from 20% in compensated liver disease to more than 80% in those patients with decompensated liver disease. Protein-calorie malnutrition (PCM) can be identified in all clinical stages but is easier observed in advanced stages of liver disease. The presence of malnutrition is associated with increased number of complications and increased short and long term mortality. Aim: to evaluate the nutritional status using of combination of BMI (Body Mass Index), TST (triceps skinfold thickness) and MAMC (mid-arm muscle circumference). Subjective Global Assessment (SGA) of nutritional status was determined for every patient. The features of subjective global assessment are history, physical evaluation and SGA rating. Based on this evaluation, patients were classified into three groups: well, moderately malnourished and severely malnourished. Material and methods: Our study was designed as a descriptive prospective analysis of patients with cirrhosis, admitted in Elias Emergency Hospital, Gastroenterology Department, during a year, January 2010-January 2011. The diagnosis of cirrhosis was based on the medical history, physical examination, biochemical findings and imagistic methods (ultrasound and / or computed tomography). A series of 176 hospitalized patients with cirrhosis, 114 (65%) male and 62 (35%) female, median age 52 (range 18-68 years). Etiology of liver disease was alcoholic in 98 (56%), hepatitis B virus in 14 (8%), HCV in 43 (24%), HBV and HDV in 10 (7%), 11 patients have other etiology. The evaluation of nutritional status was made by different methods. A detailed history was recorded with appetite, caloric intake, change in body weight. Subjective Global Assessment (SGA) of nutritional status was determined for every patient. Conclusions: Malnutrition was correlated with clinical severity of liver disease. The mild-moderate malnourished patients are 88% Child B, over 58% with viral etiology. 22% from these patients are alcoholic and 11% have Child C score (p<0.01). In severely malnourished group, 43% have alcoholic disease and 31% are Child C classification(p<0.01). Triceps skinfold thickness (mm) and mid-arm circumference(cm) decrease significantly according to the Child score, a positive correlation was found between these two parameters and the severity of cirrhosis. PMID:23483873

  4. Syncope in Patients with Pacemakers

    PubMed Central

    Sutton, Richard

    2015-01-01

    Syncope in a pacemaker patient is a serious symptom but it is rarely due a pacemaker system malfunction. Syncope occurs in about 5 % of patients paced for atrioventricular (AV) block in 5 years, 18% in those paced for sinus node disease in 10 years, 20 % of those paced for carotid sinus syndrome in 5 years and 5–55 % of those older patients paced for vasovagal syncope in 2 years. The vastly different results in vasovagal syncope depend on the results of tilt testing, where those with negative tests approach results in pacing for AV block and those with a positive tilt test show no better results than with no pacemaker. The implication of tilt results is that a hypotensive tendency is clearly demonstrated by tilt positivity pointing to syncope recurrence with hypotension. This problem may be addressed by treatment with vasoconstrictor drugs in those who are suited or, more commonly, a reduction or cessation of hypotensive therapy in hypertensive patients. Other causes of syncope such as tachyarrhythmias are rare. The clinical approach to patients who report syncope is detailed. PMID:26835124

  5. Patients' satisfaction with facial prostheses.

    PubMed

    Wondergem, Marloes; Lieben, George; Bouman, Shirley; van den Brekel, Michiel W M; Lohuis, Peter J F M

    2016-05-01

    We assessed the "impact on wellbeing" and "satisfaction" of patients who had a facial prosthesis (of the ear, nose, or orbit) fitted in The Netherlands Cancer Institute. Patients had either an adhesive-retained or an implant-retained facial prosthesis between 1951 and 2011. We did a cross-sectional survey of 104 patients, then gave a questionnaire to the final study group of 71 (68%), a year or more later. All were satisfied with their prostheses (visual analogue scale (VAS): mean (SD) 8.1(1.5). The implant-retained group were the most satisfied (p=0.022), and the adhesive-retained group felt more self-conscious (p=0.013). Three-quarters of all patients said that the prosthesis was not painful and there were no problems with the way it functioned. A well-designed facial prosthesis has obvious benefits, but there were no appreciable differences between the two groups. Each patient must make a careful decision about which type of prosthesis to choose, taking into account the quality of their remaining tissue, the site of the defect, and their general health. PMID:26508540

  6. Prescribing and partnership with patients

    PubMed Central

    Bond, Christine; Blenkinsopp, Alison; Raynor, David K

    2012-01-01

    There have been widespread changes in society and the roles of professionals. This change is also reflected in health care, where there is now acceptance of the need to involve patients in decision making. In prescribing specifically, the concordance agenda was developed alongside these initiatives to encourage improved medication taking and reduce wastage. However the extent to which these partnerships are delivered in practice remains unclear. This paper explores some of the issues to be considered when preparing patients and professionals for partnership and summarizes the limited evidence of barriers to, and benefits of, this approach. Firstly patients must be given the confidence, skills and knowledge to be partners. They need information about medicines, provided in ways known to be acceptable to them. Likewise professionals may need new skills to be partners. They need to understand the patient agenda and may need training and support to change the ways in which they consult with patients. There are also practical issues such as the perceived increase in time taken when consulting in partnership mode, room layout, computer interfaces and record keeping. Health care professionals other than doctors are also expected to behave in partnership mode, whether this is as prescribers in their own right or in supporting the prescribing of others. Whilst much has been claimed for the benefit of partnership approaches, hard evidence is limited. However whilst there is still much more to understand there will be no going back to the paternalistic model of the mid 20th century. PMID:22621201

  7. Patient blood management in Europe

    PubMed Central

    Shander, A.; Van Aken, H.; Colomina, M. J.; Gombotz, H.; Hofmann, A.; Krauspe, R.; Lasocki, S.; Richards, T.; Slappendel, R.; Spahn, D. R.

    2012-01-01

    Summary Preoperative anaemia is common in patients undergoing orthopaedic and other major surgery. Anaemia is associated with increased risks of postoperative mortality and morbidity, infectious complications, prolonged hospitalization, and a greater likelihood of allogeneic red blood cell (RBC) transfusion. Evidence of the clinical and economic disadvantages of RBC transfusion in treating perioperative anaemia has prompted recommendations for its restriction and a growing interest in approaches that rely on patients' own (rather than donor) blood. These approaches are collectively termed ‘patient blood management’ (PBM). PBM involves the use of multidisciplinary, multimodal, individualized strategies to minimize RBC transfusion with the ultimate goal of improving patient outcomes. PBM relies on approaches (pillars) that detect and treat perioperative anaemia and reduce surgical blood loss and perioperative coagulopathy to harness and optimize physiological tolerance of anaemia. After the recent resolution 63.12 of the World Health Assembly, the implementation of PBM is encouraged in all WHO member states. This new standard of care is now established in some centres in the USA and Austria, in Western Australia, and nationally in the Netherlands. However, there is a pressing need for European healthcare providers to integrate PBM strategies into routine care for patients undergoing orthopaedic and other types of surgery in order to reduce the use of unnecessary transfusions and improve the quality of care. After reviewing current PBM practices in Europe, this article offers recommendations supporting its wider implementation, focusing on anaemia management, the first of the three pillars of PBM. PMID:22628393

  8. Syncope in Patients with Pacemakers.

    PubMed

    Sutton, Richard

    2015-12-01

    Syncope in a pacemaker patient is a serious symptom but it is rarely due a pacemaker system malfunction. Syncope occurs in about 5 % of patients paced for atrioventricular (AV) block in 5 years, 18% in those paced for sinus node disease in 10 years, 20 % of those paced for carotid sinus syndrome in 5 years and 5-55 % of those older patients paced for vasovagal syncope in 2 years. The vastly different results in vasovagal syncope depend on the results of tilt testing, where those with negative tests approach results in pacing for AV block and those with a positive tilt test show no better results than with no pacemaker. The implication of tilt results is that a hypotensive tendency is clearly demonstrated by tilt positivity pointing to syncope recurrence with hypotension. This problem may be addressed by treatment with vasoconstrictor drugs in those who are suited or, more commonly, a reduction or cessation of hypotensive therapy in hypertensive patients. Other causes of syncope such as tachyarrhythmias are rare. The clinical approach to patients who report syncope is detailed. PMID:26835124

  9. Understanding patient responses to insomnia.

    PubMed

    Henry, Doug; Rosenthal, Leon; Dedrick, David; Taylor, Daniel

    2013-01-01

    To better gain insight into patient responses to insomnia, we take a medical anthropologically informed approach to patient beliefs and behaviors, particularly those related to self-diagnosis, management, help-seeking, and self-treatment of insomnia. We conducted 24 in-depth qualitative interviews in which participants were asked their beliefs about the origin of their insomnia, its anticipated course, their evaluation of symptoms, their responses, and their expectations surrounding treatment. Important and novel data were generated about patient beliefs and behaviors related to problem sleeping. Patients identified barriers to treatment, particularly those contextualized within a general social stigma and personal isolation, in which their problems sleeping were not taken seriously. The interview format was particularly conducive to making patients comfortable discussing the personal changes they made to their medically prescribed treatment plans, or supplanting their medical therapy with some kind of complimentary and alternative medicine (CAM) therapy. These are important issues in the long term management of chronic insomnia. We underscore concern about the need to evaluate the efficacy of therapies that so many people with insomnia are driven to try. PMID:23347116

  10. [COMMUNICATION WITH TERMINALLY ILL PATIENT].

    PubMed

    2014-12-01

    The communication is a essential therapeutic instrument in every process of relationship in the team patient-family, and mainly in the transmission of bad news. The communication is not just a simple transmission of information. It is a process whose goal is to enable the adaptation of the patient and family to their actual situation and where the "what", "how" and "how much do you want to know", are belonged to the own patient. Along this article, we will expose some thoughts that the team has to take into account when informing the patient. We are going to explain the SPIKES protocol, or its Spanish version EPICEE. 6-step protocol, based on those recommended by the experts to deliver bad news procedures. And finally we'll talk about the conspiracy of silence, one of the most common and difficult situations to handle in day to day due to paternalism by professionals and families, in which they prefer to hide the situation to the patient, thinking it's the best for him. PMID:26121886

  11. Creating Simulated Microgravity Patient Models

    NASA Technical Reports Server (NTRS)

    Hurst, Victor; Doerr, Harold K.; Bacal, Kira

    2004-01-01

    The Medical Operational Support Team (MOST) has been tasked by the Space and Life Sciences Directorate (SLSD) at the NASA Johnson Space Center (JSC) to integrate medical simulation into 1) medical training for ground and flight crews and into 2) evaluations of medical procedures and equipment for the International Space Station (ISS). To do this, the MOST requires patient models that represent the physiological changes observed during spaceflight. Despite the presence of physiological data collected during spaceflight, there is no defined set of parameters that illustrate or mimic a 'space normal' patient. Methods: The MOST culled space-relevant medical literature and data from clinical studies performed in microgravity environments. The areas of focus for data collection were in the fields of cardiovascular, respiratory and renal physiology. Results: The MOST developed evidence-based patient models that mimic the physiology believed to be induced by human exposure to a microgravity environment. These models have been integrated into space-relevant scenarios using a human patient simulator and ISS medical resources. Discussion: Despite the lack of a set of physiological parameters representing 'space normal,' the MOST developed space-relevant patient models that mimic microgravity-induced changes in terrestrial physiology. These models are used in clinical scenarios that will medically train flight surgeons, biomedical flight controllers (biomedical engineers; BME) and, eventually, astronaut-crew medical officers (CMO).

  12. Customer, client, consumer, recipient, or patient.

    PubMed

    Naseem, A; Balon, R; Khan, S

    2001-12-01

    Since the arrival of managed care, there has been a trend toward changing the basic terminology used to address clinicians and patients. Instead of the term "patient", third party payors frequently use terms such as "customer, client, consumer or recipient". One study demonstrated that patients prefer to be called patients. To investigate the preferred term to refer to patients and to be referred to by patients, we mailed a questionnaire to 100 physicians in four medical specialties each and to 100 psychologists. The overall response rate was 61%. Physicians overwhelmingly preferred to refer to patients by the patient's last name, their second preference was the patient's first name. Psychologists preferred to refer to the patients by first name, their second preference was the patient's last name. No group favored using terms such as client, customer, consumer, or recipient. Most physicians and psychologists preferred being referred to as doctors and nobody favored the term provider. PMID:11958366

  13. Patients as partners in innovation.

    PubMed

    Barnett, Sean J; Katz, Aviva

    2015-06-01

    As the culture of medical practice has evolved, so has the relationship between the physician and patient. This is decidedly true with regards to the introduction of innovative therapies, especially in the surgical arena. A critical challenge is identifying and defining innovative therapy. Is the proposed treatment an incremental change, a research proposal, or more commonly someplace in between? This gray area creates a transition zone commonly referred to as innovative therapy. Given the complexities of the current landscape of innovation, innovation therapy committees may provide a mechanism to help to guide both physicians and patients through such difficult topics as the process of informed consent, managing conflicts of interest, and how to evaluate the outcomes of innovative therapies. As surgical innovation remains critical to the advancement of care, it must occur in a transparent partnership with patients, under the eye of guiding entities, aimed at ultimately improving outcomes and care. PMID:25976152

  14. Diagnostic patient studies in surgery

    SciTech Connect

    Sigel, B.

    1986-01-01

    The author's approach has been to present topics that relate to general as well as some specialty applications of patient studies. These topics include all major imaging modes in use today, the commoner endoscopic procedures, and function analyses applicable to most workups of the cardiac, genitourinary, respiratory, gastrointestinal, and neurologic systems. For most of the patient studies described in this book the author has selected procedures that a surgeon is more likely to request directly. Such procedures may be new or well established. This book aims to acquaint the surgeon with some new procedures and to summarize the current status of other test that might be required during the management of a surgical patient.

  15. Dermatoglyphics in patients with schizophrenia*

    PubMed Central

    Shakibaei, Fereshteh; Asadollahi, Ghorban Ali; Tabibi, Amirpooyan

    2011-01-01

    BACKGROUND: There are controversial evidences on the association between fingerprint traits and schizophrenia. We compared fingerprint traits of patients with schizophrenia and normal individuals in Iranian population. METHODS: Finger tip dermal ridge of 290 patients with schizophrenia and 290 normal subjects were studied for four dermal traits. Data was analyzed with Pearson correlation and student's tests. RESULTS: Finger print patterns and secondary creases were not significantly different between the two groups (p > 0.05). Although mean ridge counts of left and right index fingers of the case group were greater than the control group (p < 0.05), these differences were not significant in females. CONCLUSIONS: Probably the left index ridge counts and fluctuating asymmetry in schizophrenic patients are different from those of the normal population. This difference may serve as a diagnostic biological marker for screening people susceptible to schizophrenia. Further studies are needed to determine predictive value of fingerprint trait as a biomarker for the schizophrenia. PMID:22279482

  16. Polyneuropathy in critically ill patients.

    PubMed Central

    Bolton, C F; Gilbert, J J; Hahn, A F; Sibbald, W J

    1984-01-01

    Five patients developed a severe motor and sensory polyneuropathy at the peak of critical illness (sepsis and multiorgan dysfunction complicating a variety of primary illnesses). Difficulties in weaning from the ventilator as the critical illness subsided and the development of flaccid and areflexic limbs were early clinical signs. However, electrophysiological studies, especially needle electrode examination of skeletal muscle, provided the definite evidence of polyneuropathy. The cause is uncertain, but the electrophysiological and morphological features indicate a primary axonal polyneuropathy with sparing of the central nervous system. Nutritional factors may have played a role, since the polyneuropathy improved in all five patients after total parenteral nutrition had been started, including the three patients who later died of unrelated causes. The features allow diagnosis during life, and encourage continued intensive management since recovery from the polyneuropathy may occur. Images PMID:6094735

  17. The diabetic patient in Ramadan

    PubMed Central

    Chamsi-Pasha, Hassan; Aljabri, Khalid S.

    2014-01-01

    During the month of Ramadan, all healthy, adult Muslims are required to fast from dawn to sunset. Fasting during Ramadan involves abstaining from food, water, beverages, smoking, oral drugs, and sexual intercourse. Although the Quran exempts chronically ill from fasting, many Muslims with diabetes still fast during Ramadan. Patients with diabetes who fast during the month of Ramadan can have acute complications. The risk of complications in fasting individuals with diabetes increases with longer periods of fasting. All patients with diabetes who wish to fast during Ramadan should be prepared by undergoing a medical assessment and engaging in a structured education program to undertake the fast as safely as possible. Although some guidelines do exist, there is an overwhelming need for better designed clinical trials which could provide us with evidence-based information and guidance in the management of patients with diabetes fasting Ramadan. PMID:24761380

  18. The diabetic patient in Ramadan.

    PubMed

    Chamsi-Pasha, Hassan; Aljabri, Khalid S

    2014-04-01

    During the month of Ramadan, all healthy, adult Muslims are required to fast from dawn to sunset. Fasting during Ramadan involves abstaining from food, water, beverages, smoking, oral drugs, and sexual intercourse. Although the Quran exempts chronically ill from fasting, many Muslims with diabetes still fast during Ramadan. Patients with diabetes who fast during the month of Ramadan can have acute complications. The risk of complications in fasting individuals with diabetes increases with longer periods of fasting. All patients with diabetes who wish to fast during Ramadan should be prepared by undergoing a medical assessment and engaging in a structured education program to undertake the fast as safely as possible. Although some guidelines do exist, there is an overwhelming need for better designed clinical trials which could provide us with evidence-based information and guidance in the management of patients with diabetes fasting Ramadan. PMID:24761380

  19. Dermatologic infections in cancer patients.

    PubMed

    Gandhi, Mona; Brieva, Joaquin C; Lacouture, Mario E

    2014-01-01

    Dermatologic infections are among the most commonly experienced complications of cancer and anti-cancer therapy. Alterations in host immune function secondary to the underlying malignant process and/or its treatment have been linked to an increase in the risk of infections. The skin and its appendages (i.e., hair and nails) represent the first line of defense against infectious microorganism; its dysfunction as a physical barrier and an immunologic organ in cancer patients leads to an increased susceptibility to infectious organisms. Moreover, a cancer patient's vulnerable state facilitates dissemination of infections to other sites, secondarily involving the skin. This chapter delineates dermatologic infections that are unique to cancer patients as a result of their underlying malignancies and associated comorbidities as well as those resulting from antineoplastic therapies. PMID:24706229

  20. Erythrocytosis in a scleroderma patient.

    PubMed

    Mabo, Eric; Edeki, Timi; Westney, Gloria E; Reed, James W; Guo, Xiaoqing

    2006-05-01

    A 40-year-old black male with scleroderma lung disease presented with blurry vision and headache. His presenting hemoglobin was 22.3 g/dL and his serum erythropoietin level was surprisingly low. Although nocturnal hypoxemia was evident, his daytime resting arterial oxygen saturation was normal. The patient's symptoms of hyperviscosity improved after phlebotomy, as his hemoglobin gradually decreased to 18.3 g/dL. Repeat serum erythropoietin levels were in normal and high ranges. Patients with chronic interstitial lung disease and erythrocytosis could have normoxemia at rest and a normal or low serum erythropoietin level at the peak of erythrocytosis. A repeat sampling of serum erythropoietin and monitoring of oxygen saturation during sleep and exertion may help in diagnosis. Physicians should prescribe continuous oxygen therapy for patients with chronic interstitial lung disease and erythrocytosis, even if diurnal resting hypoxemia is absent. PMID:16758101

  1. The integrated patient information system.

    PubMed

    Song, L; Ho, J; Ho, S

    1997-01-01

    The complexity and potential impact of today's health care crisis requires a comprehensive approach to problem analysis and innovative thinking in developing solution strategies. Patient care, by its information-intensive nature, involves extensive information processing for decision-making, and requires the support of an effective and efficient information system. Therefore, information Technology must play a critical part in developing any solution strategy. Blue Chip, as an industry leader in leveraging information Technology to create innovative business processes, took the challenge and found a solution to the problem of comprehensive patient information processing. Through the generous support of the Agency for Health Care Policy and Research, a branch of the US Public Health Service, the company completed research and development under two Small Business Innovative Research grants. After 3 years of intensive study, design, and development, Blue Chip created an integrated Patient information System called Care-Centric. PMID:9099031

  2. Nutritional support of hospitalized patients.

    PubMed

    Donoghue, S

    1989-05-01

    Effective nutritional support requires sound knowledge of both basic and clinical nutrition of dogs and cats as well as familiarity with products and delivery systems. Case management includes assessment of nutritional status and estimation of fuel sources. Most starved or stressed patients use fatty acids for over 70 per cent kcalME and protein for over 20 per cent kcalME. Approximate kcal needs are calculated from maintenance energy equations. Most patients respond best to enteral nutrition. Meat-based pet foods, liquid enteral products, and nutrient modules are offered in slurries or are tube-fed. Management includes careful monitoring of patients and gradual transitions to diets with more complex nutrient sources. PMID:2658286

  3. Influenza vaccination in oncology patients.

    PubMed

    Baluch, Aliyah; Pasikhova, Yanina

    2013-12-01

    It is well established that the immunological response to the seasonal trivalent influenza vaccine is attenuated in cancer patients. Furthermore, rates of seroprotection and seroconversion vary by malignancy type and are higher in patients with solid tumors, as compared either with those with hematologic malignancies or with allogeneic hematopoietic stem cell recipients. In 2009, a novel influenza strain prompted development of new vaccines and evaluation of alternative dosing strategies in an attempt to increase the rates of seroconversion in immunocompromised patients, further complicating this issue. Recent literature has demonstrated that the use of myeloablative chemotherapy regimens and biologics is correlated with decreased immunogenicity and response to influenza vaccines. Much debate still exists as to the optimal timing of influenza vaccination. Delaying vaccination from 1 week following standard chemotherapy up to 6 months following rituximab is increasingly supported by studies in this heterogeneous population. PMID:24258350

  4. Creating a patient education tool.

    PubMed

    Stonecypher, Karen

    2009-10-01

    Developing a patient education tool based on low literacy levels, behavioral theories, role modeling, and The Joint Commission's standards was the primary objective of this project. The initial goal was merely to develop a population-appropriate patient education tool. This led to a process whereby significant knowledge was gained by all to enrich overall professional development. An interdisciplinary team developed a low-literacy, self-management book for patients who had suffered a stroke. Team experts were responsible for the development of specific subject matter. Editing addressed the message, readability, typeface and font size, and charts and illustrations. Collaboration with the National Stroke Association guided the didactic pedagogical content presented. A well-known cartoonist who was a U.S. military veteran was willing to work with the team to develop the illustrations. PMID:19831328

  5. [Diet prescription in obese patients].

    PubMed

    Lesi, Carlo; Giaquinto, Ester; Valeriani, Luca; Zoni, Luisa

    2005-03-01

    Diet is a cornerstone in the treatment of obese patients with or without metabolic complications. To optimize outcome, diet treatment should always take into account factors such as the Body Mass Index, the timeframe for reaching the recommended weight loss, comorbidities (e.g. arterial hypertension, diabetes mellitus, renal disease, lipid abnormalities, hyperuricemia) and, finally, individual patient characteristics (e.g. habits, preferences, adherence capacity). Hypocaloric diets need to be adequately balanced in terms of glucides, lipids and proteins, vitamins and minerals. For these reasons the diet prescription for obese patients, particularly those with comorbidities or cardiovascular disease, should be under the guidance of expert nutrition professionals who are aware of the risks of an unbalanced diet. PMID:16128164

  6. Candidemia in major burns patients.

    PubMed

    Renau Escrig, Ana I; Salavert, Miguel; Vivó, Carmen; Cantón, Emilia; Pérez Del Caz, M Dolores; Pemán, Javier

    2016-06-01

    Major burn patients have characteristics that make them especially susceptible to candidemia, but few studies focused on this have been published. The objectives were to evaluate the epidemiological, microbiological and clinical aspects of candidemia in major burn patients, determining factors associated with a poorer prognosis and mortality. We conducted a retrospective observational study of candidemia between 1996 and 2012 in major burn patients admitted to the La Fe University Hospital, Valencia, Spain. The study included 36 episodes of candidemia in the same number of patients, 55.6% men, mean age 37.33 years and low associated comorbidity. The incidence of candidemia varied between 0.26 and 6.09 episodes/1000 days stay in the different years studied. Candida albicans was the most common species (61.1%) followed by Candida parapsilosis (27.8%). Candidemia by C. krusei, C. glabrata or C. tropicalis were all identified after 2004. Central vascular catheter (CVC) was established as a potential source of candidemia in 36.1%, followed by skin and soft tissues of thermal injury (22.2%) and urinary tract (8.3%). Fluconazole was used in 19 patients (52.7%) and its in vitro resistance rate was 13.9%. The overall mortality was 47.2%, and mortality related to candidemia was 30.6%. Factors associated with increased mortality were those related to severe infection and shock. CVC was the most usual focus of candidemia. Fluconazole was the most common antifungal drug administered. The management of candidemia in major burn patients is still a challenge. PMID:26931414

  7. Cost trajectories for cancer patients

    PubMed Central

    Wodchis, W.P.; Arthurs, E.; Khan, A.I.; Gandhi, S.; MacKinnon, M.; Sussman, J.

    2016-01-01

    Background Health care spending is known to be highly skewed, with a small subset of the population consuming a disproportionate amount of health care resources. Patients with cancer are high-cost users because of high incremental health care costs for treatment and the growing prevalence of cancer. The objectives of the present study included characterizing cancer-patient trajectories by cost, and identifying the patient and health system characteristics associated with high health system costs after cancer treatment. Methods This retrospective cohort study identified Ontario adults newly diagnosed with cancer between 1 April 2009 and 30 September 2010. Costs of health care use before, during, and after cancer episodes were used to develop trajectories of care. Descriptive analyses examined differences between the trajectories in terms of clinical and health system characteristics, and a logistic regression approach identified predictors of being a high-cost user after a cancer episode. Results Ten trajectories were developed based on whether patients were high- or low-cost users before and after their cancer episode. The most common trajectory represented patients who were low-cost in the year before cancer, survived treatment, and continued to be low-cost in the year after cancer (31.4%); stage ii cancer of the male genital system was the most common diagnosis within that trajectory. Regression analyses identified increases in age and in multimorbidity and low continuity of care as the strongest predictors of high-cost status after cancer. Conclusions Findings highlight an opportunity to proactively identify patients who might transition to high-cost status after cancer treatment and to remediate that transition. PMID:26985150

  8. Seizure Treatment in Transplant Patients

    PubMed Central

    Shepard, Paul W.

    2013-01-01

    Opinion statement Solid organ transplantation is frequently complicated by a spectrum of seizure types, including single partial-onset or generalized tonic-clonic seizures, acute repetitive seizures or status epilepticus, and sometimes the evolution of symptomatic epilepsy. There is currently no specific evidence involving the transplant patient population to guide the selection, administration, or duration of antiepileptic drug (AED) therapy, so familiarity with clinical AED pharmacology and application of sound judgment are necessary for successful patient outcomes. An initial detailed search for symptomatic seizure etiologies, including metabolic, infectious, cerebrovascular, and calcineurin inhibitor treatment-related neuro-toxic complications such as posterior reversible encephalopathy syndrome (PRES), is imperative, as underlying central nervous system disorders may impose additional serious risks to cerebral or general health if not promptly detected and appropriately treated. The mainstay for post-transplant seizure management is AED therapy directed toward the suspected seizure type. Unfavorable drug interactions could place the transplanted organ at risk, so choosing an AED with limited interaction potential is also crucial. When the transplanted organ is dysfunctional or vulnerable to rejection, AEDs without substantial hepatic metabolism are favored in post-liver transplant patients, whereas after renal transplantation, AEDs with predominantly renal elimination may require dosage adjustment to prevent adverse effects. Levetiracetam, gabapentin, pregabalin, and lacosamide are drugs of choice for treatment of partial-onset seizures in post-transplant patients given their efficacy spectrum, generally excellent tolerability, and lack of drug interaction potential. Levetiracetam is the drug of choice for primary generalized seizures in post-transplant patients. When intravenous drugs are necessary for acute seizure management, benzodiazepines and fosphenytoin are the traditional and best evidence-based options, although intravenous levetiracetam, valproate, and lacosamide are emerging options. Availability of several newer AEDs has greatly expanded the therapeutic armamentarium for safe and efficacious treatment of post-transplant seizures, but future prospective clinical trials and pharmacokinetic studies within this specific patient population are needed. PMID:22660960

  9. [Hilarein, a patient education game for kidney transplant patients].

    PubMed

    Césarini, Carole; Callens, Cécile

    2013-03-01

    At Nice university hospital, an educational board game has been designed by the kidney transplant team for patients suffering from kidney failure. Hilarein is a tool to support therapeutic education which demonstrates that it is possible to learn while having fun. PMID:23593797

  10. Patient-friendly financial options.

    PubMed

    Levin, R P

    1999-03-01

    Having flexible payment options ensures that patients will have all of the motivation and information they need to accept care in your office. The HCCC simply offers the chance to make more expensive treatment available to those who need or want it. The small cost of using a HCCC program more than pays for itself--in fact, it costs less than one-third of what you would spend if you were to bill these patients instead. Having consistent financial policies and flexible payment options can dramatically increase your office's productivity and profitability, while expressing your commitment to customer service and high-quality dentistry. PMID:10344121

  11. Patient-friendly financial options.

    PubMed

    Levin, R P

    2000-01-01

    Having flexible payment options ensures that patients will have all of the motivation and information that they need to accept care in your office. The HCCC simply offers you the chance to make more expensive treatment available to those who need or want it. The small cost of using an HCCC program more than pays for itself--in fact, it costs less than one-third of what you would spend if you were to bill these patients instead. Having consistent financial policies and flexible payment options can dramatically increase your office's productivity and profitability, while expressing your commitment to customer service and high-quality dentistry. PMID:11132302

  12. [Weight loss in cancer patients].

    PubMed

    Lordick, Florian; Hacker, Ulrich

    2016-02-01

    Cancer patients are regularly affected by malnutrition which often leads to a worsened quality of life and activity in daily living, more side effects and complications during anticancer treatment and shorter survival times. The early diagnosis and treatment of malnutrition are therefore relevant components of oncological treatment. The assessment of the nutritional status and determination of the body-mass-index should be done in every patient with cancer. The clinical examination delivers important findings and indications for malnutrition. Bioimpedance analysis can deliver additional objective information. The treatment of malnutrion should start early and follows a step-wise escalation reaching from nutritional counseling to enteral nutritional support to parenteral nutrition. PMID:26886037

  13. [Intelligent footwear for diabetic patients].

    PubMed

    Pataky, Zoltan; Grivon, Daniel; Civet, Yoan; Perriard, Yves

    2016-01-20

    The incidence of diabetic foot ulcerations and lower extremity amputations remains very high and inacceptable. The high risk of ulceration and consequent amputation is strongly related to difficulties to obtain foot off-loading, particularly on long term. Due to the complexity of their utilization, the available foot off-loading devices are underused both by health care providers and patients with very low therapeutic adherence. This article summarizes the foot off-loading in diabetic patients and describes the concept of intelligent footwear we developed, based on continuous measurements and permanent and automatic adaptations of the shoe insole's rigidity. PMID:26946791

  14. Motor planning in Parkinson patients.

    PubMed

    Sharpe, M H; Cermak, S A; Sax, D S

    1983-01-01

    This study was designed to determine if Parkinsonian patients exhibited a deficit in motor planning. Thirty adult males, 15 with Parkinson's disease and 15 normal controls, were given a gestural test which had two components. The first component required the symbolic representation of implement usage on verbal command and on imitation (representational items) and the second component required the imitation of non-symbolic hand positions (non-representational items). The results indicated that Parkinsonian patients performed at a significantly lower gestural level on the representational tasks and made significantly more spatial errors on non-representational tasks than the normal controls. PMID:6196678

  15. Ovarian stimulation in cancer patients.

    PubMed

    Cakmak, Hakan; Rosen, Mitchell P

    2013-05-01

    The patients referred for fertility preservation owing to a malignant disease do not represent the typical population of subfertile patients treated in IVF units. Cancer may affect multiple tissues throughout the body and can result in a variety of complications during controlled ovarian stimulation. Determination of the controlled ovarian stimulation protocol and gonadotropin dose for oocyte/embryo cryopreservation requires an individualized assessment. This review highlights the new protocols that are emerging to reduce time constraints and emphasizes management considerations to decrease complications. PMID:23635348

  16. Psychiatric patients' perspective: nursing uniforms.

    PubMed

    Küçük, Leyla; Çömez, Tuba; Kaçar, Selma; Sümeli, Fatma; Taşkıran, Özgül

    2015-12-01

    This paper studied and determined the importance of the design and color of selected uniforms of psychiatric nurses and evaluate their effects and implications for patients. In this descriptive study, a questionnaire developed by the researchers and a nursing uniforms catalog were used during the data collection stage of the research. There were 40.9% of the participants who told that the design and color of a nursing uniform is important and 31.5% of the participants selected white uniform color from the catalog for psychiatric nurses and 33.9% of the patients expressed that they find white nursing uniforms comforting. PMID:26577551

  17. Space Technology for Patient Monitoring

    NASA Technical Reports Server (NTRS)

    1989-01-01

    A contract for the development of an astronaut monitoring system in the early days of the space program provided Mennen Medical, Inc. with a foundation in telemetry that led to the development of a computerized medical electronic system used by hospitals. Mennen was the first company to adopt solid state design in patient monitoring and to offer multipatient telemetry monitoring. Telemetry converts instrument data to electrical signals and relays them to a remote receiver where they are displayed. From a central station, a nurse can monitor several patients. Company products include VISTA systems and Horizon 2000 Monitor.

  18. [Acute abdomen in elderly patients].

    PubMed

    Machado, M A; Pereira, E A; Loureno, J L; Branco, P D

    1990-01-01

    Seventy nine elderly patients with acute abdominal conditions were operated on in the Emergency Service of the Hospital das Clinicas of the University of So Paulo from 1981 to 1985. Total mortality rate was 58.23%. In patients with acute vascular abdominal conditions it was 100%, in inflammatory conditions 55.17% and in intestinal obstruction 47.83%. The lowest mortality was observed in traumatic conditions (27.27%). Procrastination of operations was followed by higher mortality. In operations lasting more than three hours the mortality was significantly higher than in those lasting less than three hours. Associated diseases as well as reoperations increased the postoperative mortality. PMID:2133165

  19. Managing complications in cirrhotic patients

    PubMed Central

    Angeli, Paolo; Cordoba, Juan; Farges, Oliver; Valla, Dominique

    2015-01-01

    Liver cirrhosis is a serious and potentially life-threatening condition. This life-threatening condition usually arises from complications of cirrhosis. While variceal bleeding is the most acute and probably best studied, several other complications of liver cirrhosis are more insidious in their onset but nevertheless more important for the long-term management and outcome of these patients. This review summarizes the topics discussed during the UEG-EASL Hepatology postgraduate course of the United European Gastroenterology Week 2013 and discusses emergency surgical conditions in cirrhotic patients, the management of hepatic encephalopathy, ascites and hepatorenal syndrome, coagulation disorders, and liver cancer. PMID:25653862

  20. Managing complications in cirrhotic patients.

    PubMed

    Peck-Radosavljevic, Markus; Angeli, Paolo; Cordoba, Juan; Farges, Oliver; Valla, Dominique

    2015-02-01

    Liver cirrhosis is a serious and potentially life-threatening condition. This life-threatening condition usually arises from complications of cirrhosis. While variceal bleeding is the most acute and probably best studied, several other complications of liver cirrhosis are more insidious in their onset but nevertheless more important for the long-term management and outcome of these patients. This review summarizes the topics discussed during the UEG-EASL Hepatology postgraduate course of the United European Gastroenterology Week 2013 and discusses emergency surgical conditions in cirrhotic patients, the management of hepatic encephalopathy, ascites and hepatorenal syndrome, coagulation disorders, and liver cancer. PMID:25653862

  1. Improving Patient's Primary Medication Adherence

    PubMed Central

    Leguelinel-Blache, Géraldine; Dubois, Florent; Bouvet, Sophie; Roux-Marson, Clarisse; Arnaud, Fabrice; Castelli, Christel; Ray, Valérie; Kinowski, Jean-Marie; Sotto, Albert

    2015-01-01

    Abstract Quality of transitions of care is one of the first concerns in patient safety. Redesigning the discharge process to incorporate clinical pharmacy activities could reduce the incidence of postdischarge adverse events by improving medication adherence. The present study investigated the value of pharmacist counseling sessions on primary medication adherence after hospital discharge. This study was conducted in a 1844-bed hospital in France. It was divided in an observational period and an interventional period of 3 months each. In both periods, ward-based clinical pharmacists performed medication reconciliation and inpatient follow-up. In interventional period, initial counseling and discharge counseling sessions were added to pharmaceutical care. The primary medication adherence was assessed by calling community pharmacists 7 days after patient discharge. We compared the measure of adherence between the patients from the observational period (n = 201) and the interventional period (n = 193). The rate of patients who were adherent increased from 51.0% to 66.7% between both periods (P < 0.01). When discharge counseling was performed (n = 78), this rate rose to 79.7% (P < 0.001). The multivariate regression performed on data from both periods showed that age of at least 78 years old, and 3 or less new medications on discharge order were predictive factors of adherence. New medications ordered at discharge represented 42.0% (n = 1018/2426) of all medications on discharge order. The rate of unfilled new medications decreased from 50.2% in the observational period to 32.5% in the interventional period (P < 10−7). However, patients included in the observational period were not significantly more often readmitted or visited the emergency department than the patients who experienced discharge counseling during the interventional period (45.3% vs. 46.2%; P = 0.89). This study highlights that discharge counseling sessions are essential to improve outpatients’ primary medication adherence. We identified predictive factors of primary nonadherence in order to target the most eligible patients for discharge counseling sessions. Moreover, implementation of discharge counseling could be facilitated by using Health Information Technology to adapt human resources and select patients at risk of nonadherence. PMID:26469927

  2. [Other treatments for depressive patients].

    PubMed

    Tamaoki, Toshio; Motohashi, Nobutaka

    2007-09-01

    Electroconvulsive therapy(ECT) is one of the most important methods in treating depressive patients especially who can not be improved with medication. Meta analysis shows that ECT is superior to pharmacotherapy as acute treatment for depression. ECT was invented in 1938, and it took some improvement afterwards such as development of modified ECT and introduction of brief-pulse stimulation for the purpose of reducing adverse effects. However, adverse effects such as cognitive impairment are not completely solved, and some patients do not respond to ECT. Transcranial magnetic stimulation (TMS), vagus nerve stimulation (VNS) and deep brain stimulation (DBS) are under investigation to get over the shortcomings of ECT. PMID:17876990

  3. Viagra for home care patients.

    PubMed

    Catania, P N

    1998-08-01

    In March 1998 a totally new type of therapeutic agent, sildenafil citrate (Viagra), was approved by the Food and Drug Administration for marketing in the United States as an oral tablet for erectile dysfunction in men. Extensive media coverage may have overshadowed the actual value and potential limitations of this therapeutic agent. Because sildenafil is a prescription-only medication that may be used in ambulatory and home care patients, home caregivers need to understand its intended use, mechanism and dose, and potential problems that may occur in patients who receive it. PMID:9814198

  4. Elderly patients with systemic disease.

    PubMed

    Boggs, J G

    2001-01-01

    Although the healthy elderly seizure patient can often be treated with antiepileptic drugs (AEDs) similarly to a younger counterpart, the elderly with systemic disease require a more complex approach. The interplay of multiple organ dysfunctions and multiple non-AED medications greatly increases the challenges of managing the majority of older epilepsy patients. In addition, age-related physiologic changes in hepatic and renal function, volume of distribution, and exaggerated sensitivity to side effects alter the usual "rules" for administering AEDs. Thorough knowledge of the general principles of geriatric physiology and pharmacology may predict optimal selection of initial or subsequent AED therapy in this population. PMID:11902325

  5. How to Feed Cleft Patient?

    PubMed Central

    Khan, Saima Yunus

    2013-01-01

    ABSTRACT Cleft lip and palate patients have all rights like other normal individuals, to enjoy the benefits of nourishment. Knowledge has to be there about the different feeding positions like straddle, dancer hand position along with the use of specially designed bottles and nipples. Parent's should be trained about the correct positions of feeding, in extreme of the cases in which parents are not able to follow these instructions, feeding obturators can be given. How to cite this article: Jindal MK, Khan SY. How to Feed Cleft Patient? Int J Clin Pediatr Dent 2013;6(2):100-103. PMID:25206201

  6. The patient-sensor interface

    PubMed Central

    Crockett, G. S.

    1970-01-01

    During the assessment of monitoring equipment on acute medical cases in a general ward, a quantitative investigation of technical faults revealed that 44% of these occurred at the patient-sensor interface. While the attachment of the equipment was accepted by the patient and was suitable for application by nursing staff, this degree of technical breakdown indicates that more progress is necessary in the design of this aspect of monitoring equipment before it is possible to have a reliable system. ImagesFig. 1 PMID:5476136

  7. [Electronic patient record as the tool for better patient safety].

    PubMed

    Schneider, Henning

    2015-01-01

    Recent studies indicate again that there is a deficit in the use of electronic health records (EHR) in German hospitals. Despite good arguments in favour of their use, such as the rapid availability of data, German hospitals shy away from a wider implementation. The reason is the high cost of installing and maintaining the EHRs, for the benefit is difficult to evaluate in monetary terms for the hospital. Even if a benefit can be shown it is not necessarily evident within the hospital, but manifests itself only in the health system outside. Many hospitals only manage to partly implement EHR resulting in increased documentation requirements which reverse their positive effect.In the United States, electronic medical records are also viewed in light of their positive impact on patient safety. In particular, electronic medication systems prove the benefits they can provide in the context of patient safety. As a result, financing systems have been created to promote the digitalisation of hospitals in the United States. This has led to a large increase in the use of IT systems in the United States in recent years. The Universitätsklinikum Eppendorf (UKE) introduced electronic patient records in 2009. The benefits, in particular as regards patient safety, are numerous and there are many examples to illustrate this position. These positive results are intended to demonstrate the important role EHR play in hospitals. A financing system of the ailing IT landscape based on the American model is urgently needed to benefit-especially in terms of patient safety-from electronic medical records in the hospital. PMID:25475524

  8. Determinants of Compliance among Pediatric Amblyopia Patients.

    ERIC Educational Resources Information Center

    Wolff, Hans; Juhasz, Anne McCreary

    Given the recent focus on patient responsibility for health status and improvement, it is important to understand the dynamics involved in patient compliance to treatment regimens. The determinants of patching compliance among 30 pediatric amblyopia patients and their parents were investigated by means of parent, patient, and physician…

  9. Patient safety curriculum in medical education.

    PubMed

    Lee, Young-Mee

    2009-09-01

    Since release of the Institute of Medicine (IOM) report, To Err Is Human: Building a Safer System, patient safety has emerged as a global concern in the provision of quality health care. In response to growing recognition of the importance of patient safety, some medical schools in other countries have created and/or implemented patient safety curricula. In Korea, however, patient safety medical curriculum has not been actively discussed by medical educators. The purpose of this article is to introduce patient safety concepts and the global efforts on patient safety medical education. Specifically, this article describes; 1) current concepts in patient safety, 2) global trends of patient safety movement and education, 3) contents, instructional and assessment methods of patient safety education for both undergraduate medical education and graduate medical education, suggested in the previous studies, 4) WHO Patient Safety Guide for Medical Curriculum developed by the Medical Education Team within the World Alliance for Patient Safety and 5) known barriers against patient safety education. Patient safety is a major priority for all healthcare providers. In reality, however, teaching and learning about patient safety in medical curriculum offers a challenge to all medical schools, especially, the health care environment is not favorable to physicians such as Korea. More attention and recognition about patient safety by all health personnel and medical educators is needed. In addition, the national conversation about medical errors and patient safety and how best to incorporate it to the existing curriculum should be discussed. PMID:25813311

  10. Thallium-201 stress imaging in hypertensive patients

    SciTech Connect

    Schulman, D.S.; Francis, C.K.; Black, H.R.; Wackers, F.J.

    1987-07-01

    To assess the potential effect of hypertension on the results of thallium-201 stress imaging in patients with chest pain, 272 thallium-201 stress tests performed in 133 hypertensive patients and 139 normotensive patients over a 1-year period were reviewed. Normotensive and hypertensive patients were similar in age, gender distribution, prevalence of cardiac risk factors (tobacco smoking, hyperlipidemia, and diabetes mellitus), medications, and clinical symptoms of coronary disease. Electrocardiographic criteria for left ventricular hypertrophy were present in 16 hypertensive patients. Stepwise probability analysis was used to determine the likelihood of coronary artery disease for each patient. In patients with mid to high likelihood of coronary disease (greater than 25% probability), abnormal thallium-201 stress images were present in 54 of 60 (90%) hypertensive patients compared with 51 of 64 (80%) normotensive patients. However, in 73 patients with a low likelihood of coronary disease (less than or equal to 25% probability), abnormal thallium-201 stress images were present in 21 patients (29%) of the hypertensive group compared with only 5 of 75 (7%) of the normotensive patients (p less than 0.001). These findings suggest that in patients with a mid to high likelihood of coronary artery disease, coexistent hypertension does not affect the results of thallium-201 exercise stress testing. However, in patients with a low likelihood of coronary artery disease, abnormal thallium-201 stress images are obtained more frequently in hypertensive patients than in normotensive patients.

  11. Transforming consumer health informatics through a patient work framework: connecting patients to context.

    PubMed

    Valdez, Rupa S; Holden, Richard J; Novak, Laurie L; Veinot, Tiffany C

    2015-01-01

    Designing patient-centered consumer health informatics (CHI) applications requires understanding and creating alignment with patients' and their family members' health-related activities, referred to here as 'patient work'. A patient work approach to CHI draws on medical social science and human factors engineering models and simultaneously attends to patients, their family members, activities, and context. A patient work approach extends existing approaches to CHI design that are responsive to patients' biomedical realities and personal skills and behaviors. It focuses on the embeddedness of patients' health management in larger processes and contexts and prioritizes patients' perspectives on illness management. Future research is required to advance (1) theories of patient work, (2) methods for assessing patient work, and (3) techniques for translating knowledge of patient work into CHI application design. Advancing a patient work approach within CHI is integral to developing and deploying consumer-facing technologies that are integrated with patients' everyday lives. PMID:25125685

  12. The Wired Patient: Patterns of Electronic Patient Portal Use Among Patients With Cardiac Disease or Diabetes

    PubMed Central

    Weiner, Jonathan P; Shah, Nirav R; Stewart, Walter F

    2015-01-01

    Background As providers develop an electronic health record–based infrastructure, patients are increasingly using Web portals to access their health information and participate electronically in the health care process. Little is known about how such portals are actually used. Objective In this paper, our goal was to describe the types and patterns of portal users in an integrated delivery system. Methods We analyzed 12 months of data from Web server log files on 2282 patients using a Web-based portal to their electronic health record (EHR). We obtained data for patients with cardiovascular disease and/or diabetes who had a Geisinger Clinic primary care provider and were registered “MyGeisinger” Web portal users. Hierarchical cluster analysis was applied to longitudinal data to profile users based on their frequency, intensity, and consistency of use. User types were characterized by basic demographic data from the EHR. Results We identified eight distinct portal user groups. The two largest groups (41.98%, 948/2258 and 24.84%, 561/2258) logged into the portal infrequently but had markedly different levels of engagement with their medical record. Other distinct groups were characterized by tracking biometric measures (10.54%, 238/2258), sending electronic messages to their provider (9.25%, 209/2258), preparing for an office visit (5.98%, 135/2258), and tracking laboratory results (4.16%, 94/2258). Conclusions There are naturally occurring groups of EHR Web portal users within a population of adult primary care patients with chronic conditions. More than half of the patient cohort exhibited distinct patterns of portal use linked to key features. These patterns of portal access and interaction provide insight into opportunities for electronic patient engagement strategies. PMID:25707036

  13. Poetic Interventions with Forensic Patients.

    ERIC Educational Resources Information Center

    Berger, Art; Giovan, Marti

    1990-01-01

    Describes the use of poetry, music, and creative writing with forensic patients at a state mental health institute. Demonstrates that expressive interventions were helpful in group treatment by promoting verbalization, decision making, and the recognition of personal responsibility for incarceration. (SR)

  14. Transferrin changes in haemodialysed patients.

    PubMed

    Formanowicz, Dorota; Formanowicz, Piotr

    2012-06-01

    Transferrin (Tf) is a glycoprotein responsible for iron transport in the human body. Physiologically in reaction with Concanavalin A, Tf occurs in four distinct variants Tf1, Tf2, Tf3 (apo-Tf) and Tf4. It was reported recently that Tf is changing, particularly during acute phase response, taking place among others in end-stage renal disease. In this study, we wanted to find the answer to three main questions: firstly, how Tf is changing in patients treated with maintenance haemodialysis (mHD), secondly, whether there are any Tf changes in the course of mHD treatment, and thirdly, what factors can affect Tf microheterogeneity in these patients. Studies were performed on 80 haemodialysed patients and 21 healthy volunteers. The Tf concentration was determined by the rocket immunoelectrophoresis, and its microheterogeneity was assessed by the ConA crossed immunoaffinity electrophoresis. During the annual observation of the distribution of the Tf variants, we have found both changes of the percentage contents of all Tf variants in the whole Tf concentration and a significant decrease in Tf2, Tf3 and Tf4 serum concentrations. Moreover, we found that decrease in the renal function, duration of mHD, and inflammation may contribute to these above-mentioned changes, which are probably the factors that should be taken into account when explaining the mechanisms of persistence of anaemia in haemodialysed patients. PMID:21455763

  15. Seamless patient journeys the goal.

    PubMed

    Wiegand, Chris

    2016-02-01

    Chris Wiegand, CEO of Jibestream, a software development company for digital interactive technologies with bases in Toronto and Arlington, Virginia, explains how technologies including Wi-Fi, GPS, RFID, and Bluetooth LE are enhancing wayfinding in healthcare facilities, and, in the process, simplifying the patient journey and helping reduce the stress and anxiety often associated with a visit to the hospital. PMID:27017656

  16. Patient perspectives of telemedicine quality

    PubMed Central

    LeRouge, Cynthia M; Garfield, Monica J; Hevner, Alan R

    2015-01-01

    Background The purpose of this study was to explore the quality attributes required for effective telemedicine encounters from the perspective of the patient. Methods We used a multi-method (direct observation, focus groups, survey) field study to collect data from patients who had experienced telemedicine encounters. Multi-perspectives (researcher and provider) were used to interpret a rich set of data from both a research and practice perspective. Results The result of this field study is a taxonomy of quality attributes for telemedicine service encounters that prioritizes the attributes from the patient perspective. We identify opportunities to control the level of quality for each attribute (ie, who is responsible for control of each attribute and when control can be exerted in relation to the encounter process). This analysis reveals that many quality attributes are in the hands of various stakeholders, and all attributes can be addressed proactively to some degree before the encounter begins. Conclusion Identification of the quality attributes important to a telemedicine encounter from a patient perspective enables one to better design telemedicine encounters. This preliminary work not only identifies such attributes, but also ascertains who is best able to address quality issues prior to an encounter. For practitioners, explicit representation of the quality attributes of technology-based systems and processes and insight on controlling key attributes are essential to implementation, utilization, management, and common understanding. PMID:25565781

  17. [Management of patients with stomas].

    PubMed

    Simon, Anne

    2016-01-01

    With the occurrence of an intestinal obstruction, many patients may need an intestinal stoma. This decision is often taken in an emergency context but may also be planned. The treatment will be multi-disciplinary involving the surgeon, anaesthetist, nurse, health care assistant, physiotherapist, dietician and stoma therapist. PMID:26743367

  18. Imaging Pregnant and Lactating Patients.

    PubMed

    Tirada, Nikki; Dreizin, David; Khati, Nadia J; Akin, Esma A; Zeman, Robert K

    2015-10-01

    As use of imaging in the evaluation of pregnant and lactating patients continues to increase, misperceptions of radiation and safety risks have proliferated, which has led to often unwarranted concerns among patients and clinicians. When radiologic examinations are appropriately used, the benefits derived from the information gained usually outweigh the risks. This review describes appropriateness and safety issues, estimated doses for imaging examinations that use iodizing radiation (ie, radiography, computed tomography, nuclear scintigraphy, and fluoroscopically guided interventional radiology), radiation risks to the mother and conceptus during various stages of pregnancy, and use of iodinated or gadolinium-based contrast agents and radiotracers in pregnant and lactating women. Maternal radiation risk must be weighed with the potential consequences of missing a life-threatening diagnosis such as pulmonary embolus. Fetal risks (ie, spontaneous abortion, teratogenesis, or carcinogenesis) vary with gestational age and imaging modality and should be considered in the context of the potential benefit of medically necessary diagnostic imaging. When feasible and medically indicated, modalities that do not use ionizing radiation (eg, magnetic resonance imaging) are preferred in pregnant and lactating patients. Radiologists should strive to minimize risks of radiation to the mother and fetus, counsel patients effectively, and promote a realistic understanding of risks related to imaging during pregnancy and lactation. PMID:26466183

  19. [Maintaining patients' autonomy at home].

    PubMed

    Niang, Bénédicte; Coudre, Jean Pierre

    2015-01-01

    To maintain the flow of hospital discharges, the patient's return home with support from a home nursing service is important. If any difficulties are identified, there are various programmes or good practices which can be put into place. The future law on adapting society to ageing also comprises a scheme combining home assistance and nursing care. PMID:26144953

  20. Odontogenic lesions in pediatric patients.

    PubMed

    Fang, Qi-Gen; Shi, Shuang; Sun, Chang-Fu

    2014-05-01

    The purpose was to evaluate our 20-year experience of pediatric odontogenic lesions. Pediatric patients with a diagnosis of odontogenic lesion were identified. Three hundred ten patients were odontogenic; dentigerous cyst was seen in 62.0% of the cases. Most (70.2%) of them occurred in mixed dentition period, and it had a male preponderance. Odontogenic keratocystic tumor occurred in the permanent dentition period. It had an equal site distribution. Odontoma was seen in 20.0% of the cases. Its site of predilection was the mandible. Ameloblastoma was the most common odontogenic tumor. Most of the cases occurred in the permanent dentition period. It affected the male and female equally. Calcifying epithelioma odontogenic tumor was seen in 11.8% of the cases. All the lesions occurred in the primary dentition period. It had no sex or site preponderance. Myxoma was seen in 3.6% of the cases. It was most common in the permanent dentition period, and it was more frequent in the male. Iliac crest bone graft was successfully performed in 28 patients, postoperative infection occurred in 2 patients, and no donor-site dysfunctions were reported. The observed differences in lesion type and distribution in this study compared with previous researches may be attributable to genetic and geographic variation in the populations studied. Iliac crest bone graft was suggested for pediatric mandible reconstruction. PMID:24785745