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Sample records for gm1 gangliosidosis patients

  1. 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

  2. GM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings.

    PubMed

    Caciotti, Anna; Garman, Scott C; Rivera-Coln, Yadilette; Procopio, Elena; Catarzi, Serena; Ferri, Lorenzo; Guido, Carmen; Martelli, Paola; Parini, Rossella; Antuzzi, Daniela; Battini, Roberta; Sibilio, Michela; Simonati, Alessandro; Fontana, Elena; Salviati, Alessandro; Akinci, Gulcin; Cereda, Cristina; Dionisi-Vici, Carlo; Deodato, Francesca; d'Amico, Adele; d'Azzo, Alessandra; Bertini, Enrico; Filocamo, Mirella; Scarpa, Maurizio; di Rocco, Maja; Tifft, Cynthia J; Ciani, Federica; Gasperini, Serena; Pasquini, Elisabetta; Guerrini, Renzo; Donati, Maria Alice; Morrone, Amelia

    2011-07-01

    GM1 gangliosidosis and Morquio B syndrome, both arising from beta-galactosidase (GLB1) deficiency, are very rare lysosomal storage diseases with an incidence of about 1:100,000-1:200,000 live births worldwide. Here we report the beta-galactosidase gene (GLB1) mutation analysis of 21 unrelated GM1 gangliosidosis patients, and of 4 Morquio B patients, of whom two are brothers. Clinical features of the patients were collected and compared with those in literature. In silico analyses were performed by standard alignments tools and by an improved version of GLB1 three-dimensional models. The analysed cohort includes remarkable cases. One patient with GM1 gangliosidosis had a triple X syndrome. One patient with juvenile GM1 gangliosidosis was homozygous for a mutation previously identified in Morquio type B. A patient with infantile GM1 gangliosidosis carried a complex GLB1 allele harbouring two genetic variants leading to p.R68W and p.R109W amino acid changes, in trans with the known p.R148C mutation. Molecular analysis showed 27 mutations, 9 of which are new: 5 missense, 3 microdeletions and a nonsense mutation. We also identified four new genetic variants with a predicted polymorphic nature that was further investigated by in silico analyses. Three-dimensional structural analysis of GLB1 homology models including the new missense mutations and the p.R68W and p.R109W amino acid changes showed that all the amino acid replacements affected the resulting protein structures in different ways, from changes in polarity to folding alterations. Genetic and clinical associations led us to undertake a critical review of the classifications of late-onset GM1 gangliosidosis and Morquio B disease. PMID:21497194

  3. Structural bases of GM1 gangliosidosis and Morquio B disease.

    PubMed

    Morita, Mizuki; Saito, Seiji; Ikeda, Kazuyoshi; Ohno, Kazuki; Sugawara, Kanako; Suzuki, Toshihiro; Togawa, Tadayasu; Sakuraba, Hitoshi

    2009-09-01

    Allelic mutations of the lysosomal beta-galactosidase gene cause heterogeneous clinical phenotypes, such as GM1 gangliosidosis and Morquio B disease, the former being further classified into three variants, namely infantile, juvenile and adult forms; and heterogeneous biochemical phenotypes were shown in these forms. We tried to elucidate the bases of these diseases from a structural viewpoint. We first constructed a three-dimensional structural model of human beta-galactosidase by means of homology modeling. The human beta-galactosidase consists of three domains, such as, a TIM barrel fold domain, which functions as a catalytic domain, and two galactose-binding domain-like fold domains. We then constructed structural models of representative mutant beta-galactosidase proteins (G123R, R201C, I51T and Y83H) and predicted the structural change associated with each phenotype by calculating the number of affected atoms, determining the root-mean-square deviation and the solvent-accessible surface area, and by color imaging. The results show that there is a good correlation between the structural changes caused by amino-acid substitutions in the beta-galactosidase molecule, as well as biochemical and clinical phenotypes in these representative cases. Protein structural study is useful for elucidating the bases of these diseases. PMID:19644515

  4. 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

  5. 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

  6. Extensive and Progressing Congenital Dermal Melanocytosis Leading to Diagnosis of GM1 Gangliosidosis.

    PubMed

    Vedak, Priyanka; Sells, Ryan; De Souza, Aieska; Hoang, Mai P; Kroshinsky, Daniela

    2015-01-01

    Congenital dermal melanocytosis (CDM) is a birthmark composed of macular blue-grey hyperpigmentation commonly observed in the lumbosacral region of infants. Generally resolving by childhood, it is traditionally considered a benign condition, but it may be a sign of underlying lysosomal storage disease. We report a case of biopsy-confirmed CDM in a 2-month-old girl of Brazilian descent later diagnosed with infantile GM1 gangliosidosis. PMID:26337817

  7. Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis.

    PubMed

    Heinecke, Karie A; Luoma, Adrienne; d'Azzo, Alessandra; Kirschner, Daniel A; Seyfried, Thomas N

    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 ?-galactosidase (?-gal) +/? and ?-gal -/- mice, a model of GM1gangliosidosis. Optic nerve weight was lower in the ?-gal -/- mice than in unaffected ?-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 ?-gal -/- mice. The content of myelin-enriched cerebrosides, sulfatides, and plasmalogen ethanolamines was significantly lower in optic nerve of ?-gal -/- mice than in ?-gal +/? mice; however, cholesteryl esters were enriched in the ?-gal -/- mice. No major abnormalities in these lipids were detected in the sciatic nerve of the ?-gal -/- mice. The abnormalities in GM1 and myelin lipids in optic nerve of ?-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 ?-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 ?-gal -/- mice is likely due to the greater glycolipid storage in optic nerve. PMID:25694553

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

    PubMed

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

    2013-03-01

    Lysosomal ?-galactosidase (?-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 ?-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 ?-Gal. In vitro, 6S-NBI-DGJ had the ability to inhibit the activity of human ?-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 ?-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 ?-Gal mutants. PMID:23337983

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

    PubMed Central

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

    2013-01-01

    Lysosomal ?-galactosidase (?-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 ?-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 ?-Gal. In vitro, 6S-NBI-DGJ had the ability to inhibit the activity of human ?-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 ?-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 ?-Gal mutants. PMID:23337983

  10. 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

  11. 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

  12. Canine GM1-gangliosidosis. A clinical, morphologic, histochemical, and biochemical comparison of two different models.

    PubMed Central

    Alroy, J.; Orgad, U.; DeGasperi, R.; Richard, R.; Warren, C. D.; Knowles, K.; Thalhammer, J. G.; Raghavan, S. S.

    1992-01-01

    The clinical, morphologic, histochemical, and biochemical features of GM1-gangliosidosis in two canine models, English Springer Spaniel (ESS) and Portuguese Water Dog (PWD), have been compared. The disease onset, its clinical course, and survival period of the affected dogs were similar in both models. Skeletal dysplasia was noted radiographically at 2 months of age, whereas at 4 1/2 months of age there was progressive neurologic impairment. However, dwarfism and coarse facial features were seen only in ESS. Both models had similar deficiency in activity of lysosomal beta-galactosidase, but possessed a normal protein activator for GM1-beta-galactosidase. Both models stored GM1-ganglioside, asialo-GM1, and oligosaccharides in brain. Furthermore, only the PWD stored glycoproteins containing polylactosaminoglycans in visceral organs, and neither model stored them in the brain. Morphologically, both models demonstrated similar storage material in multiple tissues and cell types. The ultrastructure of the storage material was cell-type specific and identical in both models. However, some differences in the lectin staining pattern were noted. Our clinical, biochemical, and histochemical findings indicate that PWD and ESS may represent two different mutations of the beta-galactosidase gene. Moreover, the authors conclude that it is difficult, and inappropriate, to apply the human classification of GM1-gangliosidosis (i.e. infantile, juvenile, and adult forms) to these canine models. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 Figure 14 Figure 15 PMID:1546746

  13. 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

  14. GM1 gangliosidosis in a Japanese domestic cat: a new variant identified in Hokkaido, Japan.

    PubMed

    Ueno, Hiroshi; Yamato, Osamu; Sugiura, Takeshi; Kohyama, Moeko; Yabuki, Akira; Miyoshi, Kenjiro; Matsuda, Kazuya; Uchide, Tsuyoshi

    2016-02-01

    A male Japanese domestic cat with retarded growth in Hokkaido, Japan, showed progressive motor dysfunction, such as ataxia starting at 3 months of age and tremors, visual disorder and seizure after 4 months of age. Finally, the cat died of neurological deterioration at 9 months of age. Approximately half of the peripheral blood lymphocytes had multiple abnormal vacuoles. Magnetic resonance imaging showed bisymmetrical hyperintensity in the white matter of the parietal and occipital lobes in the forebrain on T2-weighted and fluid-attenuated inversion recovery images, and mild encephalatrophy of the olfactory bulbs and temporal lobes. The activity of lysosomal acid ?-galactosidase in leukocytes was negligible, resulting in the biochemical diagnosis of GM1 gangliosidosis. Histologically, swollen neurons characterized by accumulation of pale, slightly granular cytoplasmic materials were observed throughout the central nervous system. Dysmyelination or demyelination and gemistocytic astrocytosis were observed in the white matter. Ultrastructually, membranous cytoplasmic bodies were detected in the lysosomes of neurons. However, genetic analysis did not identify the c.1448G>C mutation, which is the single known mutation of feline GM1 gangliosidosis, suggesting that the cat was affected with a new variant of the feline disease. PMID:26234889

  15. Hepatic storage of oligosaccharides and glycolipids in a cat affected with GM1 gangliosidosis.

    PubMed Central

    Holmes, E W; O'Brien, J S

    1978-01-01

    1. Glycopeptides and glycolipids were isolated from normal cat liver and liver from a cat affected with GM1 gangliosidosis. 2. Bio-Gel P-6 chromatography of the crude glycopeptide fractions demonstrated three major peaks of hexose-containing compounds that were greatly increased in the mutant liver sample; these peaks contained oligosaccharides that comprised over 2% of the liver wet weight. 3. Two of the major pathological oligosaccharides, GP5 and GP6, were purified by chromatography on charcoal/Celite and Sephadex G-25. Oligosaccharides GP5 and GP6 had apparent mol.wts. of 1800 +/- 200 and 1350+/-200 respectively, and contained galactose, mannose and N-acetylglucosamine in molar proportions of 2.0:3.1:4.1 (GP5) and 1.0:2.2:2.7 (GP6). Periodate oxidation studies demonstrated the presence of galactose in a non-reducing terminal position. 4. The neutral glycolipid fraction from the mutant cat liver has a 1.3-fold increase in hexose content accompanied by an increased concentration of asialo-(ganglioside GM1). 5. There was a 2-fold increase of gangliosides in the mutant cat liver compared with normal cats. Ganglioside GM1 and a compound tentatively identified as N-glycolloyl-(ganglioside GM1) were the major glycolipids accumulated. Images Fig. 2. Fig. 5. PMID:217359

  16. 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

  17. 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

  18. A Duplication in the Canine ?-Galactosidase Gene GLB1 Causes Exon Skipping and GM1-Gangliosidosis in Alaskan Huskies

    PubMed Central

    Kreutzer, Robert; Leeb, Tosso; Mller, Gundi; Moritz, Andreas; Baumgrtner, Wolfgang

    2005-01-01

    GM1-gangliosidosis is a lysosomal storage disease that is inherited as an autosomal recessive disorder, predominantly caused by structural defects in the ?-galactosidase gene (GLB1). The molecular cause of GM1-gangliosidosis in Alaskan huskies was investigated and a novel 19-bp duplication in exon 15 of the GLB1 gene was identified. The duplication comprised positions +1688+1706 of the GLB1 cDNA. It partially disrupted a potential exon splicing enhancer (ESE), leading to exon skipping in a fraction of the transcripts. Thus, the mutation caused the expression of two different mRNAs from the mutant allele. One transcript contained the complete exon 15 with the 19-bp duplication, while the other transcript lacked exon 15. In the transcript containing exon 15 with the 19-bp duplication a premature termination codon (PTC) appeared, but due to its localization in the last exon of canine GLB1, nonsense-mediated RNA decay (NMD) did not occur. As a consequence of these molecular events two different truncated GLB1 proteins are predicted to be expressed from the mutant GLB1 allele. In heterozygous carrier animals the wild-type allele produces sufficient amounts of the active enzyme to prevent clinical signs of disease. In affected homozygous dogs no functional GLB1 is synthesized and GM1-gangliosidosis occurs. PMID:15944348

  19. 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

  20. 1-Deoxy-d-galactonojirimycins with dansyl capped N-substituents as ?-galactosidase inhibitors and potential probes for GM1 gangliosidosis affected cell lines

    PubMed Central

    Frhlich, Richard F.G.; Furneaux, Richard H.; Mahuran, Don J.; Saf, Robert; Sttz, Arnold E.; Tropak, Michael B.; Wicki, Jacqueline; Withers, Stephen G.; Wrodnigg, Tanja M.

    2011-01-01

    Two simple and reliably accessible intermediates, N-carboxypentyl- and N-aminohexyl-1-deoxy-d-galactonojirimycin were employed for the synthesis of a set of terminally N-dansyl substituted derivatives. Reaction of the terminal carboxylic acid of N-carboxypentyl-1-deoxy-d-galactonojirimycin with N-dansyl-1,6-diaminohexane provided the chain-extended fluorescent derivative. Employing bis(6-dansylaminohexyl)amine, the corresponding branched di-N-dansyl compound was obtained. Partially protected N-aminohexyl-1-deoxy-d-galactonojirimycin served as intermediate for two additional chain-extended fluorescent 1-deoxy-d-galactonojirimycin (1-DGJ) derivatives featuring terminal dansyl groups in the N-alkyl substituent. These new compounds are strong inhibitors of d-galactosidases and may serve as leads en route to pharmacological chaperones for GM1-gangliosidosis. PMID:21645885

  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. Genetics Home Reference: GM1 gangliosidosis

    MedlinePLUS

    ... lose the skills they had previously acquired (developmentally regress) and may develop an exaggerated startle reaction to ... gingival ; gums ; hepatosplenomegaly ; hypertrophy ; inherited ; involuntary ; juvenile ; recessive ; regress ; regression ; retina ; spectrum ; tissue ; toxic You may find ...

  3. Genetics Home Reference: GM1 gangliosidosis

    MedlinePLUS

    ... retina) gradually deteriorates. An eye abnormality called a cherry-red spot, which can be identified with an ... experience developmental regression but usually do not have cherry-red spots, distinctive facial features, or enlarged organs. ...

  4. Alterations in cholesterol and ganglioside GM1 content of lipid rafts in platelets from patients with Alzheimer disease.

    PubMed

    Liu, Li; Zhang, Ke; Tan, Liang; Chen, Yu-Hua; Cao, Yun-Peng

    2015-01-01

    The aim of this study was to investigate the changes in the protein, cholesterol, and ganglioside GM1 content of lipid rafts in platelets from patients with Alzheimer disease (AD), and identify potential blood biomarkers of the disease. A total of 31 Chinese patients with AD and 31 aged-matched control subjects were selected. Lipid rafts were isolated from platelets using Optiprep gradient centrifugation. The protein content of lipid rafts was evaluated using Micro BCA assay, the cholesterol content using molecular probes, ganglioside GM1 content using colorimetry and dot-blotting analysis. The results showed that the cholesterol and ganglioside GM1 content of lipid rafts from platelets was significantly higher in patients with AD than aged-matched control subjects, whereas the protein content of lipid rafts did not show any differences between the 2 groups. These results indicate that the increases in the cholesterol and ganglioside GM1 content of lipid rafts from the platelets of patients with AD might serve as a biochemical adjunct to the clinical diagnosis of AD. PMID:24759545

  5. Ganglioside-monosialic acid (GM1) prevents oxaliplatin-induced peripheral neurotoxicity in patients with gastrointestinal tumors

    PubMed Central

    2013-01-01

    Background Oxaliplatin, an effective antineoplastic agent againstgastrointestinal tumors, can cause severe peripheral neurotoxicity, which seriously limits its clinical application. To date, there are no effective treatments for this complication. Ganglioside-monosialic acid (GM1) has been shown to protect neurons against injuries and degeneration. The aim of this study was to evaluate the effects of GM1 on preventing oxaliplatin-induced neurotoxicity in patients with gastrointestinal tumors. Methods In this study, 120 patients with gastrointestinal tumors were enrolled, andthey received the treatment of XELOX (oxaliplatin and capecitabine) and FOLFOX4 (oxaliplatin, leukovolin and 5-fluorouracil). The patients were randomly divided into two groups, the experimental group and control group, with60 patients ineach. On the day chemotherapy was initiated, the experimental group received GM1 intravenously (100 mg once daily) for 3 days, while no neuroprotective agents were applied in the control group. The incidence rates and classification of neurotoxicity in the two groups were evaluated and the differences between the two groups were examined. Furthermore, whether GM1 affected the therapeutic effects of chemotherapy was also examined. Results The grade of neurotoxicity in the experimental group was significantly lower than in the control group (P<0.05, MannWhitney U test). The probability of occurrence of low-grade neurotoxicity (grade 0 and 1) in the experimental group was higher than that in the control group (logistic ordinal regression); whereas the probability of occurrence of high-grade neurotoxicity (grade 2 and 3) in the experimental group was lower than in the control group (logistic ordinal regression). Conclusion The data suggested that GM1 could reduce the grade of oxaliplatin-induced neurotoxicity and was an effective neuroprotective agent against oxaliplatin-induced high-grade neurotoxicity in patients with gastrointestinal tumors. PMID:23351188

  6. A Randomized, Controlled, Delayed Start Trial of GM1 Ganglioside in Treated Parkinsons Disease Patients

    PubMed Central

    Schneider, Jay S.; Gollomp, Stephen M.; Sendek, Stephanie; Colcher, Amy; Cambi, Franca; Du, Wei

    2012-01-01

    The present single center, double-blind, delayed start study was conducted to examine possible symptomatic and disease-modifying effects of GM1 ganglioside in Parkinsons disease (PD). Seventy-seven subjects with PD were randomly assigned to receive GM1 for 120 weeks (early-start group) or placebo for 24 weeks followed by GM1 for 96 weeks (delayed-start group). Washout evaluations occurred at 1 and 2 years after the end of treatment. Seventeen additional subjects who received standard-of-care were followed for comparative information about disease progression. Primary outcome was change from baseline Unified Parkinsons Disease Rating Scale (UPDRS) motor scores. At week 24, the early-start group had significant improvement in UPDRS motor scores vs. a significant worsening of scores in the delayed-start group. The early-start group also showed a sustained benefit vs. the delayed-start group at week 72 and at week 120. Both groups had significant symptom worsening during washout. This study provides evidence that GM1 use for 24 weeks was superior to placebo for improving motor symptoms and that extended GM1 use (up to 120 weeks) resulted in a lower than expected rate of symptom progression. The data from this small study suggest that GM1 may have symptomatic and potentially disease modifying effects on PD. PMID:23199590

  7. The AB-variant of GM2-gangliosidosis. Clinical, biochemical, and pathological studies of two patients.

    PubMed

    Goldman, J E; Yamanaka, T; Rapin, I; Adachi, M; Suzuki, K; Suzuki, K

    1980-01-01

    Clinical, neuropathological, and biochemical studies are reported in two children with the AB-variant of GM2-gangliosidosis. One patient had become symptomatic by 1--1.5 years, initially showing cerebellar signs, and then progressive psychomotor retardation, with hypotonia, spasticity, dementia, and macular cherry red spots, until death at the age of 4.5 years. The second patient showed an earlier onset of retardation and a more rapidly progressive course. At postmortem, the brains were of normal or near normal weights and displayed grossly only mild cerebral cortical and cerebellar atrophy, and mild pallor or attenuation of the white matter. Neuronal storage was widespread throughout the CNS, and both neurons and glia contained a variety of abnormal, membranous inclusions. Visceral organs were not involved. Ganglioside sialic acid was increased several fold in gray matter, with GM2 the predominant ganglioside species. N-acetyl-beta-glucosaminidase activities in serum, leukocytes, fibroblasts, and postmortem gray matter, assayed with an artificial, fluorogenic substrate, were normal, as were activities of other lysosomal hydrolases. PMID:6255724

  8. 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 ?-galactosidase (?-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.

  9. GM1 ganglioside in the treatment of Parkinson's disease.

    PubMed

    Schneider, J S

    1998-06-19

    Since the early 1980s, numerous studies have been reported by laboratories around the world documenting the beneficial effects of GM1 ganglioside treatment on the damaged dopamine system in various animal and in vitro models. Based on the strength of these data, the first clinical studies designed to assess the efficacy and safety of chronic GM1 use in the treatment of Parkinson's disease were performed. In a double-blind placebo-controlled study, significant improvements in GM1-treated patients were demonstrated in clinical motor ratings, timed tests of motor function, activities of daily living, and some aspects of neuropsychological functioning. Patients who have elected to continue using GM1 in an open extension trial have either continued to improve over time or have shown initial functional improvements and their disease has remained stable (i.e., no symptom progression) after two years. These results suggest that long-term use of GM1 is safe and may work to partially reverse the degenerative process in established Parkinson's disease patients. PMID:9668369

  10. 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

  11. 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

  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-ganglioside accumulation at the mitochondria-associated ER membranes links ER stress to Ca(2+)-dependent mitochondrial apoptosis.

    PubMed

    Sano, Renata; Annunziata, Ida; Patterson, Annette; Moshiach, Simon; Gomero, Elida; Opferman, Joseph; Forte, Michael; d'Azzo, Alessandra

    2009-11-13

    Mitochondria-associated ER membranes, or MAMs, define the sites of endoplasmic reticulum/mitochondria juxtaposition that control Ca(2+) flux between these organelles. We found that in a mouse model of the human lysosomal storage disease GM1-gangliosidosis, GM1-ganglioside accumulates in the glycosphingolipid-enriched microdomain (GEM) fractions of MAMs, where it interacts with the phosphorylated form of IP3 receptor-1, influencing the activity of this channel. Ca(2+) depleted from the ER is then taken up by the mitochondria, leading to Ca(2+) overload in this organelle. The latter induces mitochondrial membrane permeabilization (MMP), opening of the permeability transition pore, and activation of the mitochondrial apoptotic pathway. This study identifies the GEMs as the sites of Ca(2+) diffusion between the ER and the mitochondria. We propose a new mechanism of Ca(2+)-mediated apoptotic signaling whereby GM1 accumulation at the GEMs alters Ca(2+) dynamics and acts as a molecular effector of both ER stress-induced and mitochondria-mediated apoptosis of neuronal cells. PMID:19917257

  14. Amyloid ?-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 ?-peptide (A?) with the phospholipid bilayer. In particular, the ganglioside GM1, present with cholesterol in lipid rafts, seems to be able to initiate A? aggregation on membrane. We studied the thermodynamic and structural effects of the presence of GM1 on the interaction between A? and liposomes, a good membrane model system. Isothermal Titration Calorimetry highlighted the importance of the presence of GM1 in recruiting monomeric A? 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 A?. The results suggest that the interaction with GM1 brings to insertion of A? 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

  15. Carbohydrate mimicry between human ganglioside GM1 and Campylobacter jejuni lipooligosaccharide causes GuillainBarr syndrome

    PubMed Central

    Yuki, Nobuhiro; Susuki, Keiichiro; Koga, Michiaki; Nishimoto, Yukihiro; Odaka, Masaaki; Hirata, Koichi; Taguchi, Kyoji; Miyatake, Tadashi; Furukawa, Koichi; Kobata, Tetsuji; Yamada, Mitsunori

    2004-01-01

    Molecular mimicry between microbial and self-components is postulated as the mechanism that accounts for the antigen and tissue specificity of immune responses in postinfectious autoimmune diseases. Little direct evidence exists, and research in this area has focused principally on T cell-mediated, antipeptide responses, rather than on humoral responses to carbohydrate structures. GuillainBarr syndrome, the most frequent cause of acute neuromuscular paralysis, occurs 12 wk after various infections, in particular, Campylobacter jejuni enteritis. Carbohydrate mimicry [Gal?13GalNAc?14(NeuAc?23)Gal?1-] between the bacterial lipooligosaccharide and human GM1 ganglioside is seen as having relevance to the pathogenesis of GuillainBarr syndrome, and conclusive evidence is reported here. On sensitization with C. jejuni lipooligosaccharide, rabbits developed anti-GM1 IgG antibody and flaccid limb weakness. Paralyzed rabbits had pathological changes in their peripheral nerves identical with those present in GuillainBarr syndrome. Immunization of mice with the lipooligosaccharide generated a mAb that reacted with GM1 and bound to human peripheral nerves. The mAb and anti-GM1 IgG from patients with GuillainBarr syndrome did not induce paralysis but blocked muscle action potentials in a musclespinal cord coculture, indicating that anti-GM1 antibody can cause muscle weakness. These findings show that carbohydrate mimicry is an important cause of autoimmune neuropathy. PMID:15277677

  16. 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

  17. 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

  18. A double-blind, placebo-controlled crossover study of ganglioside GM1 treatment for Alzheimer's disease.

    PubMed

    Flicker, C; Ferris, S H; Kalkstein, D; Serby, M

    1994-01-01

    In a double-blind trial, 12 patients with probable Alzheimer's disease were treated for 6 weeks with placebo and 6 weeks with monosialoganglioside (GM1) (100 mg/day i.m.), which potentiates the actions of nerve growth factor. GM1 failed to produce significant improvement in patients' cognitive test performance, suggesting that this is not a viable approach to the treatment of cognitive deterioration in Alzheimer's disease. PMID:8267111

  19. 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

  20. Exogenous GM1 ganglioside increases accumbal BDNF levels in rats.

    PubMed

    Valdomero, Anala; Perondi, Mara C; Orsingher, Otto A; Cuadra, Gabriel R

    2015-02-01

    Gangliosides are compounds that are abundant throughout the CNS, participating actively in neuroplasticity. We previously described that exogenous GM1 ganglioside pretreatment enhances the rewarding properties of cocaine, evidenced by a lower number of sessions and/or dosage necessary to induce conditioned place preference (CPP). Since GM1 pretreatment did not modify cocaine's pharmacokinetic parameters, we suspected that the increased rewarding effect found might be mediated by BDNF, a neurotrophic factor closely related to cocaine addiction. This study was performed to investigate the possibility that GM1 may induce changes in BDNF levels in the nucleus accumbens (NAc), a core structure in the brain's reward circuitry, of rats submitted to three conditioning sessions with cocaine (10 mg/kg, i.p.). The results demonstrate that GM1 administration, which showed no rewarding effect by itself in the CPP, induced a significant increase of BDNF protein levels in the NAc, which may account for the increased rewarding effect of cocaine shown in the CPP paradigm. PMID:25453740

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

    MedlinePLUS

    ... and may include treatment providers. Genetic Testing Registry: Tay-Sachs disease, variant AB You might also find information on ... AB variant Activator Deficiency/GM2 Gangliosidosis Activator-deficient Tay-Sachs disease GM2 Activator Deficiency Disease GM2 gangliosidosis, type AB ...

  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. 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

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

    PubMed

    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-Barr 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

  5. 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

  6. Heat-Labile Enterotoxin: Beyond GM1 Binding

    PubMed Central

    Mudrak, Benjamin; Kuehn, Meta J.

    2010-01-01

    Enterotoxigenic Escherichia coli (ETEC) is a significant source of morbidity and mortality worldwide. One major virulence factor released by ETEC is the heat-labile enterotoxin LT, which is structurally and functionally similar to cholera toxin. LT consists of five B subunits carrying a single catalytically active A subunit. LTB binds the monosialoganglioside GM1, the toxins host receptor, but interactions with A-type blood sugars and E. coli lipopolysaccharide have also been identified within the past decade. Here, we review the regulation, assembly, and binding properties of the LT B-subunit pentamer and discuss the possible roles of its numerous molecular interactions. PMID:22069646

  7. 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

  8. A Korean patient with Morquio B disease with a novel c.13_14insA mutation in the GLB1 gene.

    PubMed

    Sohn, Young Bae; Park, Hyung-Doo; Park, Sung Won; Kim, Se-Hwa; Cho, Sung-Yoon; Ko, Ah-Ra; Ki, Chang-Seok; Yeau, Sunghee; Jin, Dong-Kyu

    2012-01-01

    Mutations in the GLB1 gene, which encodes acid ?-galactosidase, can result in two disease phenotypes: GM1-gangliosidosis (MIM #230500) and Morquio B disease (Mucopolysaccharidosis type IVB, MIM #253010). Morquio B disease occurs much more infrequently than GM1-gangliodosis and is characterized by severe skeletal manifestations (dysostosis multiplex) without central nervous system involvement. Here, we report the first known Korean patient with Morquio B disease. A 7-year-old boy presented with severe progressive skeletal dysplasia including scoliosis, contractures of the elbows, xenu valgum, funnel chest, and trigger thumb requiring surgical intervention. The patient had normal neurological functions and mental status when evaluated by pediatric neurologists. The patient's urinary glycosaminoglycans, measured by the cetylpyridinium chloride (CPC) precipitation test, were 252.8 CPC unit/g creatinine (reference range < 175). Thin layer chromatography of urine showed a keratan sulfate band. Enzyme activity of ?-galactosidase in leukocytes was 1.15 nmol/hr/mg protein (reference range 78.1-117.7; 1-1.5% of normal). The patient had compound heterozygous mutations of the GLB1 gene: c.13_14insA (p.L5HfsX29), which was reported in a patient with infantile GM1 gangliosidosis with the near-complete absence of enzyme activity, and c.367G>A (p.G123R), which is a novel frame-shift mutation. In summary, we report the first known Korean patient with Morquio B disease and a novel mutation (c.13_14insA of GLB1). PMID:22371915

  9. 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.

  10. GM1 ganglioside induces vasodilation and increases catalase content in the brain.

    PubMed

    Furian, Ana Flvia; Oliveira, Mauro Schneider; Royes, Luiz Fernando Freire; Fiorenza, Natlia Gindri; Fighera, Michele Rechia; Myskiw, Jociane Carvalho; Weiblen, Rudi; Rubin, Maribel Antonello; Frussa-Filho, Roberto; Mello, Carlos Fernando

    2007-09-15

    Monosialoganglioside (GM1) is a glycosphingolipid present in most cell membranes that displays antioxidant and neuroprotective properties. GM1 increases catalase activity in cerebral cortices in vivo, but the mechanisms underlying this effect of GM1 are not known. In the current study we investigated the effect of GM1 (50 mg/kg, ip) on the content of hemoglobin and catalase activity of hippocampus, cortex, and striatum of rats. GM1 administration increased catalase activity and hemoglobin content in brain samples after 30 min, but had no effect on blood catalase activity. GM1-induced increase in catalase activity was abolished by brain perfusion with heparinized saline. Brain catalase activity in the absence of blood, estimated by regression analysis of data from perfused and nonperfused animals, was not altered by the systemic injection of GM1. Moreover, the addition of GM1 (30 or 100 microM) did not increase catalase activity in slices of cerebral cortex in situ, further suggesting that blood circulation is required for this effect. The GM1-induced vasodilation was confirmed in vivo, because the systemic injection of GM1 (50 mg/kg, ip) increased (1.2-1.6 times) the width of pial vessels. PMID:17697937

  11. 5-Fluoro derivatives of 4-epi-isofagomine as d-galactosidase inhibitors and potential pharmacological chaperones for GM1-gangliosidosis as well as Fabry's disease.

    PubMed

    Thonhofer, Martin; Gonzalez Santana, Andres; Fischer, Roland; Torvisco Gomez, Ana; Saf, Robert; Schalli, Michael; Sttz, Arnold E; Withers, Stephen G

    2016-02-01

    Electrophilic fluorination of an exocyclic methoxymethylene enol ether derived from N-tert-butyloxycarbonyl-1,5-dideoxy-1,5-imino-3,4-O-isopropylidene-d-erythro-pent-2-ulose (11) provided the 5-fluoro derivative of the powerful ?-galactosidase inhibitor 4-epi-isofagomine (8). This structural alteration, in combination with N-alkylation, led to considerably improved ?-galactosidase selectivity. New compounds may serve as leads en route to new pharmacological chaperones for Fabry's disease. PMID:26717544

  12. 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

  13. GM1 Clustering Inhibits Cholera Toxin Binding in Supported Phospholipid Membranes

    PubMed Central

    Shi, Jinjun; Yang, Tinglu; Kataoka, Sho; Zhang, Yanjie; Diaz, Arnaldo J.; Cremer, Paul S.

    2012-01-01

    The present studies explore multivalent ligandreceptor interactions between pentameric cholera toxin B subunits (CTB) and the corresponding membrane ligand, ganglioside GM1. CTB binding was monitored on supported phospholipid bilayers coated on the walls and floors of microfluidic channels. Measurements were made by total internal reflection fluorescence microscopy (TIRFM). Apparent dissociation constants were extracted by fitting the binding data to both the HillWaud and Langmuir adsorption isotherm equations. Studies of the effect of ligand density on multivalent CTBGM1 interactions revealed that binding weakened with increasing GM1 density from 0.02 mol % to 10.0 mol %. Such a result could be explained by the clustering of GM1 on the supported phospholipid membranes, which in turn inhibited the binding of CTB. Atomic force microscopy (AFM) experiments directly verified GM1 clustering within the supported POPC bilayers. PMID:17429973

  14. 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

  15. Decoupling polarization of the Golgi apparatus and GM1 in the plasma membrane.

    PubMed

    Bisel, Blaine; Calamai, Martino; Vanzi, Francesco; Pavone, Francesco Saverio

    2013-01-01

    Cell polarization is a process of coordinated cellular rearrangements that prepare the cell for migration. GM1 is synthesized in the Golgi apparatus and localized in membrane microdomains that appear at the leading edge of polarized cells, but the mechanism by which GM1 accumulates asymmetrically is unknown. The Golgi apparatus itself becomes oriented toward the leading edge during cell polarization, which is thought to contribute to plasma membrane asymmetry. Using quantitative image analysis techniques, we measure the extent of polarization of the Golgi apparatus and GM1 in the plasma membrane simultaneously in individual cells subject to a wound assay. We find that GM1 polarization starts just 10 min after stimulation with growth factors, while Golgi apparatus polarization takes 30 min. Drugs that block Golgi polarization or function have no effect on GM1 polarization, and, conversely, inhibiting GM1 polarization does not affect Golgi apparatus polarization. Evaluation of Golgi apparatus and GM1 polarization in single cells reveals no correlation between the two events. Our results indicate that Golgi apparatus and GM1 polarization are controlled by distinct intracellular cascades involving the Ras/Raf/MEK/ERK and the PI3K/Akt/mTOR pathways, respectively. Analysis of cell migration and invasion suggest that MEK/ERK activation is crucial for two dimensional migration, while PI3K activation drives three dimensional invasion, and no cumulative effect is observed from blocking both simultaneously. The independent biochemical control of GM1 polarity by PI3K and Golgi apparatus polarity by MEK/ERK may act synergistically to regulate and reinforce directional selection in cell migration. PMID:24312472

  16. 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.

  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. 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

  19. 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

  20. 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

  1. 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

  2. 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

  3. 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

  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. 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

  6. 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

  7. 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

  8. GM1-Modified Lipoprotein-like Nanoparticle: Multifunctional Nanoplatform for the Combination Therapy of Alzheimer's Disease.

    PubMed

    Huang, Meng; Hu, Meng; Song, Qingxiang; Song, Huahua; Huang, Jialin; Gu, Xiao; Wang, Xiaolin; Chen, Jun; Kang, Ting; Feng, Xingye; Jiang, Di; Zheng, Gang; Chen, Hongzhuan; Gao, Xiaoling

    2015-11-24

    Alzheimer's disease (AD) exerts a heavy health burden for modern society and has a complicated pathological background. The accumulation of extracellular ?-amyloid (A?) is crucial in AD pathogenesis, and A?-initiated secondary pathological processes could independently lead to neuronal degeneration and pathogenesis in AD. Thus, the development of combination therapeutics that can not only accelerate A? clearance but also simultaneously protect neurons or inhibit other subsequent pathological cascade represents a promising strategy for AD intervention. Here, we designed a nanostructure, monosialotetrahexosylganglioside (GM1)-modified reconstituted high density lipoprotein (GM1-rHDL), that possesses antibody-like high binding affinity to A?, facilitates A? degradation by microglia, and A? efflux across the blood-brain barrier (BBB), displays high brain biodistribution efficiency following intranasal administration, and simultaneously allows the efficient loading of a neuroprotective peptide, NAP, as a nanoparticulate drug delivery system for the combination therapy of AD. The resulting multifunctional nanostructure, ?NAP-GM1-rHDL, was found to be able to protect neurons from A?1-42 oligomer/glutamic acid-induced cell toxicity better than GM1-rHDL in vitro and reduced A? deposition, ameliorated neurologic changes, and rescued memory loss more efficiently than both ?NAP solution and GM1-rHDL in AD model mice following intranasal administration with no observable cytotoxicity noted. Taken together, this work presents direct experimental evidence of the rational design of a biomimetic nanostructure to serve as a safe and efficient multifunctional nanoplatform for the combination therapy of AD. PMID:26440073

  9. 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

  10. 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

  11. 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

  12. 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.

  13. Relative Roles of GM1 Ganglioside, N-Acylneuraminic Acids, and ?2?1 Integrin in Mediating Rotavirus Infection

    PubMed Central

    Fleming, Fiona E.; Bhm, Raphael; Dang, Vi T.; Holloway, Gavan; Haselhorst, Thomas; Madge, Paul D.; Deveryshetty, Jaigeeth; Yu, Xing; Blanchard, Helen; von Itzstein, Mark

    2014-01-01

    ABSTRACT N-acetyl- and N-glycolylneuraminic acids (Sia) and ?2?1 integrin are frequently used by rotaviruses as cellular receptors through recognition by virion spike protein VP4. The VP4 subunit VP8*, derived from Wa rotavirus, binds the internal N-acetylneuraminic acid on ganglioside GM1. Wa infection is increased by enhanced internal Sia access following terminal Sia removal from main glycan chains with sialidase. The GM1 ligand cholera toxin B (CTB) reduces Wa infectivity. Here, we found sialidase treatment increased cellular GM1 availability and the infectivity of several other human (including RV-3) and animal rotaviruses, typically rendering them susceptible to methyl ?-d-N-acetylneuraminide treatment, but did not alter ?2?1 usage. CTB reduced the infectivity of these viruses. Aceramido-GM1 inhibited Wa and RV-3 infectivity in untreated and sialidase-treated cells, and GM1 supplementation increased their infectivity, demonstrating the importance of GM1 for infection. Wa recognition of ?2?1 and internal Sia were at least partially independent. Rotavirus usage of GM1 was mapped to VP4 using virus reassortants, and RV-3 VP8* bound aceramido-GM1 by saturation transfer difference nuclear magnetic resonance (STD NMR). Most rotaviruses recognizing terminal Sia did not use GM1, including RRV. RRV VP8* interacted minimally with aceramido-GM1 by STD NMR. Unusually, TFR-41 rotavirus infectivity depended upon terminal Sia and GM1. Competition of CTB, Sia, and/or aceramido-GM1 with cell binding by VP8* from representative rotaviruses showed that rotavirus Sia and GM1 preferences resulted from VP8*-cell binding. Our major finding is that infection by human rotaviruses of commonly occurring VP4 serotypes involves VP8* binding to cell surface GM1 glycan, typically including the internal N-acetylneuraminic acid. IMPORTANCE Rotaviruses, the major cause of severe infantile gastroenteritis, recognize cell surface receptors through virus spike protein VP4. Several animal rotaviruses are known to bind sialic acids at the termini of main carbohydrate chains. Conversely, only a single human rotavirus is known to bind sialic acid. Interestingly, VP4 of this rotavirus bound to sialic acid that forms a branch on the main carbohydrate chain of the GM1 ganglioside. Here, we use several techniques to demonstrate that other human rotaviruses exhibit similar GM1 usage properties. Furthermore, binding by VP4 to cell surface GM1, involving branched sialic acid recognition, is shown to facilitate infection. In contrast, most animal rotaviruses that bind terminal sialic acids did not utilize GM1 for VP4 cell binding or infection. These studies support a significant role for GM1 in mediating host cell invasion by human rotaviruses. PMID:24501414

  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. 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.

  16. 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.

  17. Neurophysiological and immunohistochemical studies of IgG anti-GM1 monoclonal antibody on neuromuscular transmission: effects in rat neuromuscular junctions.

    PubMed

    Hotta, Sayako; Nagaoka, Takumi; Taguchi, Kyoji; Nakatani, Yoshihiko; Utsnomiya, Iku; Masuda, Yutaka; Abe, Kenji; Yuki, Nobuhiro

    2014-02-01

    Guillain-Barr syndrome, which is a variant of acute inflammatory neuropathy, is associated with anti-GM1 antibodies and causes ataxia. We investigated the effects of IgG anti-GM1 monoclonal antibody (IgG anti-GM1 mAb) on spontaneous muscle action potentials in a rat spinal cord-muscle co-culture system and the localization of IgG anti-GM1 mAb binding in the rat hemi-diaphragm. The frequency of spontaneous muscle action potentials in innervated muscle cells was acutely inhibited by IgG anti-GM1 mAb. When cultures were pretreated with GM2 synthase antisense oligodeoxynucleotide, IgG anti-GM1 mAb failed to inhibit spontaneous muscle action potentials, demonstrating the importance of the GM1 epitope in the action of IgG anti-GM1 mAb. Immunohistochemistry of rat hemi-diaphragm showed that IgG anti-GM1 mAb binding overlapped with neurofilament 200 (NF200) antibodies staining, but not ?-bungarotoxin (?-BuTx) staining, demonstrating that IgG anti-GM1 mAb was localized at the presynaptic nerve terminal. IgG anti-GM1 mAb binding overlapped with syntaxin antibody and S-100 antibody in the nerve terminal. After collagenase treatment, IgG anti-GM1 mAb and NF200 antibodies did not show staining, but ?-BuTx selectively stained the hemi-diaphragm. IgG anti-GM1 mAb binds to the presynaptic nerve terminal of neuromuscular junctions. Therefore, we suggest that the inhibitory effect of IgG anti-GM1 mAb on spontaneous muscle action potentials is related to the GM1 epitope in presynaptic motor nerve terminals at the NMJs. PMID:23820959

  18. Binding, Conformational Transition and Dimerization of Amyloid-? Peptide on GM1-Containing Ternary Membrane: Insights from Molecular Dynamics Simulation

    PubMed Central

    Manna, Moutusi; Mukhopadhyay, Chaitali

    2013-01-01

    Interactions of amyloid-? (A?) with neuronal membrane are associated with the progression of Alzheimers disease (AD). Ganglioside GM1 has been shown to promote the structural conversion of A? and increase the rate of peptide aggregation; but the exact nature of interaction driving theses processes remains to be explored. In this work, we have carried out atomistic-scale computer simulations (totaling 2.65 s) to investigate the behavior of A? monomer and dimers in GM1-containing raft-like membrane. The oligosaccharide head-group of GM1 was observed to act as scaffold for A?-binding through sugar-specific interactions. Starting from the initial helical peptide conformation, a ?-hairpin motif was formed at the C-terminus of the GM1-bound A?-monomer; that didnt appear in absence of GM1 (both in fluid POPC and liquid-ordered cholesterol/POPC bilayers and also in aqueous medium) within the simulation time span. For A?-dimers, the ?-structure was further enhanced by peptide-peptide interactions, which might influence the propensity of A? to aggregate into higher-ordered structures. The salt-bridges and inter-peptide hydrogen bonds were found to account for dimer stability. We observed spontaneous formation of intra-peptide D23-K28 salt-bridge and a turn at V24GSN27 region - long been accepted as characteristic structural-motifs for amyloid self-assembly. Altogether, our results provide atomistic details of A?-GM1 and A?-A? interactions and demonstrate their importance in the early-stages of GM1-mediated A?-oligomerisation on membrane surface. PMID:23951128

  19. [Gangliosides GM1 and GD1a modulate inflammatory effect of bacterial lipopolysaccharide in epithelial cells].

    PubMed

    Nikolaeva, S D; Bakhteeva, V T; Avrova, N F; Parnova, R G

    2013-12-01

    It is known that exogenous gangliosides (GL) inhibit acute inflammatory signals in different cells induced by Escherichia coli lipopolysaccharide (LPS). Until now the mechanisms underlying their effect are unknown. We hypothesize that the anti-inflammatory effect of GL is caused by their ability to modify TLR4 translocation into the lipid rafts. To test this hypothesis, we studied the effect of exogenous GL on LPS-induced inflammatory reactions associated with increased nitric oxide and prostaglandin E2 (PGE2) production in epithelial cells isolated from the frog Rana temporia urinary bladder. It was shown that preincubation of cells with GM1 and GD1a in the concentration range from 100 nm to 50 ?M reduced the effect of 25 ?g/ml LPS E. coli on the increase of NO and PGE2 production. The effect of LPS was also eliminated in the presence of polymyxin B, capable to interact with lipid A in LPS molecule, which makes it inaccessible for binding to TLR4. The subcellular fractionation of epithelial cells in the sucrose density gradient in combination with immunoblotting revealed that LPS stimulates translocation of TLR4 into the lipid rafts in the cytoplasmic membrane. Preincubation of cells with GM1 or GD1a at concentration 20 ?M completely eliminated the effect of LPS. A similar effect was revealed with 1 mM methyl-?-cyclodextrin, a classical destructor of the lipid rafts. The results indicate the existence of a previously unknown mechanism of the anti-inflammatory effect of exogenous GL associated with their ability to interfere with LPS-induced translocation of TLR4 into the lipid rafts preventing LPS signal transduction. It is assumed that the observed effect of GL is based on their incorporation into cytoplasmic membrane and modification of the lipid rafts organization. PMID:25464770

  20. Receptor-specific large-scale purification of cholera toxin on silica beads derivatized with lysoGM1 ganglioside.

    PubMed

    Tayot, J L; Holmgren, J; Svennerholm, L; Lindblad, M; Tardy, M

    1981-01-01

    1. A receptor-specific affinity chromatographic method for large-scale purification of cholera toxin is described. The receptor ganglioside for cholera toxin, GM1, is hydrolysed to lysoGM1 which is then covalently coupled, via stabilized Schiff's bases, to porous silica beads (Spherosil) onto which a layer of DEAE-dextran has been adsorbed and cross-linked before coupling. Columns of these Spherosil-DEAE-dextran-lysoGM1 beads, in contrast to particles derivatized with lysoGA1, bound the cholera toxin of Vibrio cholerae culture filtrates, after which the toxin could be eluted with the aid of an acid citrate buffer (pH 2.8). 2. The toxin-binding capacity was directly proportional to the amount of lysoGM1 in the column: 2.3 mg/mu mol lysoGM1. The yield of purified toxin after acid elution and pH neutralization was essentially quantitative (83-107%). 3. The affinity-purified toxin contained less than 5% impurities, but consisted of a mixture of predominantly intact holotoxin and B subunit protomer which could readily be separated by gel filtration on Sephadex G-100. 4. Scaling up of the technique was possible: a 1 kg column enabled us to treat 1000-1 cultures of V. cholerae and thus to isolate 20 g of cholera toxin per cycle. PMID:6258916

  1. 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 ?- D-Gal p-(1?3)-?-D-Gal pNAc-(1?4)[?-D-Neu5Ac-(2?3)]-?- D-Gal p-(1?4)-?-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.

  2. 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 mNm(-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

  3. 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

  4. 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

  5. GPR37 protein trafficking to the plasma membrane regulated by prosaposin and GM1 gangliosides promotes cell viability.

    PubMed

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

    2014-02-21

    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 GPR37(tGFP) surface density and decreased cell viability in catecholaminergic N2a cells. We found that GPR37(tGFP) 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-?-cyclodextrin or cholesterol oxidase. Moreover, complex formation between GPR37(tGFP) 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

  6. 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

  7. Tissue-Resident NK Cells Mediate Ischemic Kidney Injury and Are Not Depleted by Anti-Asialo-GM1 Antibody.

    PubMed

    Victorino, Francisco; Sojka, Dorothy K; Brodsky, Kelley S; McNamee, Eoin N; Masterson, Joanne C; Homann, Dirk; Yokoyama, Wayne M; Eltzschig, Holger K; Clambey, Eric T

    2015-11-15

    NK cells are innate lymphoid cells important for immune surveillance, identifying and responding to stress, infection, and/or transformation. Whereas conventional NK (cNK) cells circulate systemically, many NK cells reside in tissues where they appear to be poised to locally regulate tissue function. In the present study, we tested the contribution of tissue-resident NK (trNK) cells to tissue homeostasis by studying ischemic injury in the mouse kidney. Parabiosis experiments demonstrate that the kidney contains a significant fraction of trNK cells under homeostatic conditions. Kidney trNK cells developed independent of NFIL3 and T-bet, and they expressed a distinct cell surface phenotype as compared with cNK cells. Among these, trNK cells had reduced asialo-GM1 (AsGM1) expression relative to cNK cells, a phenotype observed in trNK cells across multiple organs and mouse strains. Strikingly, anti-AsGM1 Ab treatment, commonly used as an NK cell-depleting regimen, resulted in a robust and selective depletion of cNKs, leaving trNKs largely intact. Using this differential depletion, we tested the relative contribution of cNK and trNK cells in ischemic kidney injury. Whereas anti-NK1.1 Ab effectively depleted both trNK and cNK cells and protected against ischemic/reperfusion injury, anti-AsGM1 Ab preferentially depleted cNK cells and failed to protect against injury. These data demonstrate unanticipated specificity of anti-AsGM1 Ab depletion on NK cell subsets and reveal a new approach to study the contributions of cNK and trNK cells in vivo. In total, these data demonstrate that trNK cells play a key role in modulating local responses to ischemic tissue injury in the kidney and potentially other organs. PMID:26453755

  8. 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-? (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 C?-1 (PLC?-1), a TrkA adaptor protein, via phosphorylation. U73122, an endogenous PLC inhibitor, and siRNA for PLC?-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 PLC?-1 (second step) leads to alterations in membrane dynamics, followed by strong phosphorylation of TrkA. PMID:25910247

  9. The sialic acid residue of exogenous GM1 ganglioside is recycled for biosynthesis of sialoglycoconjugates in rat liver.

    PubMed Central

    Ghidoni, R; Trinchera, M; Sonnino, S; Chigorno, V; Tettamanti, G

    1987-01-01

    In order to assess metabolic recycling of sialic acid, GM1 ganglioside [nomenclature of Svennerholm (1964) J. Lipid. Res. 5, 145-155; IUPAC-IUB Recommendations (1977) Lipids 12, 455-468], 14C-radiolabelled at the acetyl group of sialic acid, was intravenously injected into Wistar rats, and the presence of radioactive sialic acid in liver sialoglycolipids (gangliosides) and sialoglycoproteins was ascertained. A time-course study (20 min-72 h) showed that the radioactivity present in the liver distributed in the following fractions, with reciprocal proportion varying with time: the protein (glycoprotein) fraction, the ganglioside fraction and the diffusible fraction, which contained low-Mr compounds, including sialic acid. Ganglioside-linked radioactivity gradually decreased with time; protein-linked radioactivity appeared soon after injection (20 min), reached a maximum around 20 h, then slowly diminished; diffusible radioactivity provided a sharp peak at 4 h, then rapidly decreased till disappearing after 40 h. The behaviour of bound radioactivity in the individual liver gangliosides was as follows: (a) rapid diminution with time in GM1, although with a lower rate at the longer times after injection; (b) early appearance (20 min) with a peak at 1 h, followed by continuous diminution, in GM2; (c) early appearance (20 min), peak at 1 h, diminution till 4 h, followed by a plateau, in GM3; (d) appearance at 60 min, maximum around 40 h and slow diminution thereafter, in GD1a, GD1b and GT1b. A detailed study, accomplished at 40 h after injection, demonstrated that almost all radioactivity present in the protein fraction was released by mild acid treatment and recovered in purified sialic acid; most of radioactive glycoprotein-bound sialic acid was releasable by sialidase action. In addition, the radioactivity present in the different gangliosides was exclusively carried by sialic acid and present in both sialidase-resistant and sialidase-labile residues. Only in the case of GD1a was the specific radioactivity of sialidase-resistant sialic acid superior to that of sialidase-releasable sialic acid. The results obtained lead to the following conclusions: (a) radioactive GM3 and GM2 were produced by degradation of GM1 taken up; GM3 originated partly by a process of neosynthesis; (b) radioactive GM1 consisted in part of residual exogenous GM1 and in part of a neosynthetized product; (c) radioactive GD1a originated in part by direct sialylation of GM1 taken up and in part by a neosynthetic process; (d) radioactive GD1b and GT1b resulted only from neosynthesis.(ABSTRACT TRUNCATED AT 400 WORDS) Images Fig. 3. Fig. 5. PMID:3689344

  10. 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.

  11. 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

  12. Effects of MK-801 and ganglioside GM1 on postischemic prostanoid release and hippocampal lesion in gerbil brain.

    PubMed

    Lazarewicz, J W; Sali?ska, E; Speina, E; Gadamski, R

    1994-01-01

    In this study Mongolian gerbils were submitted to a normothermic bilateral carotid ligation lasting 5 min. A noncompetitive antagonist of NMDA receptors, MK-801, 0.8 mg/kg, was injected i.p. 30 min before ischemia, or the ganglioside GM1, 30 mg/kg, was given i.p. for 3 days, twice a day. The morphology of the hippocampal CA1 neurones and the brain content of cyclooxygenase metabolites of arachidonic acid: prostaglandin 6-keto PGF1 alpha and thromboxane Tx B2 were studied. Untreated ischemia induced the accumulation in brain of the 6-keto PGF1 alpha and Tx B2 immunoreactive materials, and resulted in a lesion of 70% of CA1 neurones. In the MK-801- and GM1-pretreated groups the postischemic levels of Tx B2 were significantly decreased. However MK-801 and GM1 did not prevent damage to the CA1 neurones in gerbils normothermic after ischemia, whereas a partial neuroprotection was observed in hypothermic, MK-801 treated gerbils. The results of this study indicate that NMDA receptors may participate in the mechanism of postischemic release of eicosanoids in brain. They also confirm a potential modulatory role of gangliosides. These results are discussed in terms of the involvement of cyclooxygenase metabolites of arachidonic acid in the mechanism of a selective delayed neuronal damage to the hippocampus CA1 after ischemia. PMID:7887181

  13. Cholesterol accelerates the binding of Alzheimer's ?-amyloid peptide to ganglioside GM1 through a universal hydrogen-bond-dependent sterol tuning of glycolipid conformation

    PubMed Central

    Fantini, Jacques; Yahi, Nouara; Garmy, Nicolas

    2013-01-01

    Age-related alterations of membrane lipids in brain cell membranes together with high blood cholesterol are considered as major risk factors for Alzheimer's disease. Yet the molecular mechanisms by which these factors increase Alzheimer's risk are mostly unknown. In lipid raft domains of the plasma membrane, neurotoxic Alzheimer's beta-amyloid (Abeta) peptides interact with both cholesterol and ganglioside GM1. Recent data also suggested that cholesterol could stimulate the binding of Abeta to GM1 through conformational modulation of the ganglioside headgroup. Here we used a combination of physicochemical and molecular modeling approaches to decipher the mechanisms of cholesterol-assisted binding of Abeta to GM1. With the aim of decoupling the effect of cholesterol on GM1 from direct Abeta-cholesterol interactions, we designed a minimal peptide (Abeta5-16) containing the GM1-binding domain but lacking the amino acid residues involved in cholesterol recognition. Using the Langmuir technique, we showed that cholesterol (but not phosphatidylcholine or sphingomyelin) significantly accelerates the interaction of Abeta5-16 with GM1. Molecular dynamics simulations suggested that Abeta5-16 interacts with a cholesterol-stabilized dimer of GM1. The main structural effect of cholesterol is to establish a hydrogen-bond between its own OH group and the glycosidic-bond linking ceramide to the glycone part of GM1, thereby inducing a tilt in the glycolipid headgroup. This fine conformational tuning stabilizes the active conformation of the GM1 dimer whose headgroups, oriented in two opposite directions, form a chalice-shaped receptacle for Abeta. These data give new mechanistic insights into the stimulatory effect of cholesterol on Abeta/GM1 interactions. They also support the emerging concept that cholesterol is a universal modulator of protein-glycolipid interactions in the broader context of membrane recognition processes. PMID:23772214

  14. Single molecule experiments emphasize GM1 as a key player of the different cytotoxicity of structurally distinct A?1-42 oligomers.

    PubMed

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

    2016-02-01

    It is well established that cytotoxic A? oligomers are the key factor that triggers the initial tissue and cell modifications eventually culminating in the development of Alzheimer's disease. A?1-42 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

  15. Ganglioside GM1 prevents N-methyl-D-aspartate neurotoxicity in rabbit hippocampus in vivo. Effects on calcium homeostasis.

    PubMed

    Lazarewicz, J W; Sali?ska, E; Matyja, E

    1995-01-01

    Microdialysis was used to apply 1 mM N-methyl-D-aspartate (NMDA) for 20 min to the hippocampus of rabbits, control and pre-treated with GM1 ganglioside (im injections of 30 mg/kg for 3 d, twice a day). Concentrations of ionized Ca2+ and 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha)-immunoreactive material in the dialyzates and 45Ca and [14C]sucrose efflux from the prelabeled hippocampus were determined. After 24 h, the morphology of the hippocampal neurons was examined. In control animals, the application of NMDA resulted in 25% decrease in Ca2+ concentration and in 1000% increase in 6-keto PGF 1 alpha concentration in the dialyzates. A 30% decrease in 45Ca efflux was accompanied by 20% increase in [14C]sucrose efflux, reflecting a corresponding reduction of the extracellular space volume. A degeneration of CA1 pyramidal neurons in the vicinity of a microdialysis probe was observed. In GM1-treated rabbits the NMDA-induced decrease in Ca2+ concentrations in the dialyzates was not reduced significantly, whereas a 70% stimulation of 45Ca efflux was noted, with a concomitant 40% reduction of 6-keto-PG F1 alpha release. NMDA-evoked increase in [14C]sucrose efflux did not differ from the control. In these animals CA1 neurons were well preserved. These results indicate that the pretreatment with GM1 results in activation of calcium extrusion from the NMDA-stimulated rabbit hippocampal neurons that alleviates destabilization of calcium homeostasis and reduces NMDA-evoked neuronal injury. PMID:7632320

  16. Excretion into feces of asialo GM1 in the murine digestive tract and Lactobacillus johnsonii exhibiting binding ability toward asialo GM1. A possible role of epithelial glycolipids in the discharge of intestinal bacteria.

    PubMed

    Iwamori, Masao; Iwamori, Yuriko; Adachi, Shigeki; Nomura, Taisei

    2011-01-01

    In the digestive tract of mice (HR-1, 5 months old, ?), asialo GM1 (GA1) exhibiting receptor activity toward several intestinal bacteria was preferentially expressed in the small intestine. Also, less than 10% of GA1 in the small intestine was converted into fucosylated and sulfated derivatives, but it was completely converted to fucosyl GA1 (FGA1) in the stomach, cecum and colon. Among the lipid components in these tissues, glycolipids other than Forssman antigen and cholesterol sulfate (CS) were present in the digestive tract contents. However, sulfated GA1, sulfatide and fucosyl GM1 in the gastro-intestinal contents were not present in the cecal and colonic contents, in which the major glycolipids were ceramide monohexoside (CMH), GA1 and FGA1. The total amount of GA1 in the whole contents was 20% of that in the tissues. Thus, glycolipids were stable during the process of digestion, and excreted from the body together with cholesterol and CS. On the other hand, Lactobacillus johnsonii (LJ), whose receptor is GA1, was detected in the cecal and colonic contents on sequential analysis of 16S-ribosomal RNA and TLC-immunostaining of antigenic glycolipids with anti-LJ antiserum. LJ was found to comprise 20% of the total bacteria cultured in the lactobacillus medium under aerobic conditions, and to be present in the cecal and colonic contents, 9.8??10(7) cells versus 37 ?g GA1 and 1.4??10(8) cells versus 49 ?g GA1, respectively. Thus, GA1 in the contents might facilitate the discharge of intestinal bacteria by becoming attached them to prevent their irregular diffusion. PMID:21174147

  17. 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

  18. 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

  19. Carbohydrate-based anti-adhesive inhibition of Vibrio cholerae toxin binding to GM1-OS immobilized into artificial planar lipid membranes.

    PubMed

    Sinclair, Haydn R; Kemp, Fred; Slegte, Jaap de; Gibson, Glenn R; Rastall, Robert A

    2009-10-12

    We have studied 'food grade' sialyloligosaccharides (SOS) as anti-adhesive drugs or receptor analogues, since the terminal sialic acid residue has already been shown to contribute significantly to the adhesion and pathogenesis of the Vibrio cholerae toxin (Ctx). GM1-oligosaccharide (GM1-OS) was immobilized into a supporting POPC lipid bilayer onto a surface plasmon resonance (SPR) chip, and the interaction between uninhibited Ctx and GM1-OS-POPC was measured. SOS inhibited 94.7% of the Ctx binding to GM1-OS-POPC at 10mg/mL. The SOS EC(50) value of 5.521mg/mL is high compared with 0.2811microg/mL (182.5rhoM or 1.825x10(-10)M) for GM1-OS. The commercially available sialyloligosaccharide (SOS) mixture Sunsial E((R)) is impure, containing one monosialylated and two disialylated oligosaccharides in the ratio 9.6%, 6.5% and 17.5%, respectively, and 66.4% protein. However, these inexpensive food-grade molecules are derived from egg yolk and could be used to fortify conventional food additives, by way of emulsifiers, sweeteners and/or preservatives. The work further supports our hypothesis that SOS could be a promising natural anti-adhesive glycomimetic against Ctx and prevent subsequent onset of disease. PMID:19665695

  20. Raman spectroscopy for detecting supported planar lipid bilayers composed of ganglioside-GM1/sphingomyelin/cholesterol in the presence of amyloid-?.

    PubMed

    Hu, Zhiping; Wang, Xiaoli; Wang, Weirong; Zhang, Zhenlong; Gao, Huiping; Mao, Yanli

    2015-09-21

    The aggregation and fibril formation of amyloid ?(A?) peptides onto a ganglioside-GM1-containing lipid membrane is a cause of neurodegenerative diseases. The mechanism of the initial binding and the conformational changes of A? on the membrane should be clarified. Fluorescence microscopy and Raman spectroscopy have been performed to investigate the supporting planar lipid bilayers (SPBs) composed of ganglioside-GM1, sphingomyelin and cholesterol. It is demonstrated that the SPBs are in a liquid-crystalline state when placed on mica, and increasing the amount of ganglioside-GM1 can decrease the lateral interaction between the acyl chains of the SPBs. It has been found that A?(1-40) initially interacts with the galactose ring of the ganglioside-GM1 head group, leading to its binding and gradual aggregation on the membrane surface. The obvious change observed in Raman spectroscopy in the ?(C-H) region confirms that the hydrophobic C-terminal of A?(1-40) inserts itself into the hydrophobic part of the SPBs. The Raman data indicate that ?-helix and ?-sheet structures of A?(1-40) increase and coexist over longer time frames. Based on these results, a model was proposed to describe the mechanism of the conformational changes and the aggregation of A?(1-40) that are mediated by ganglioside-GM1-containing SPBs. PMID:26256454

  1. GM2 gangliosidosis in Saudi Arabia: multiple mutations and considerations for future carrier screening.

    PubMed

    Kaya, Namik; Al-Owain, Mohammad; Abudheim, Nada; Al-Zahrani, Jawaher; Colak, Dilek; Al-Sayed, Moeen; Milanlioglu, Aysel; Ozand, Pinar T; Alkuraya, Fowzan S

    2011-06-01

    The GM2 gangliosidose, Tay-Sachs and Sandhoff diseases, are a class of lysosomal storage diseases in which relentless neurodegeneration results in devastating neurological disability and premature death. Primary prevention is the most effective intervention since no effective therapy is currently available. An extremely successful model for the prevention of GM2 gangliosidosis in the Ashkenazi Jewish community is largely attributable to the very limited number of founder mutations in that population. Consistent with our previous observation of allelic heterogeneity in consanguineous populations, we show here that these diseases are largely caused by private mutations which present a major obstacle in replicating the Ashkenazi success story. Alternative solutions are proposed which can also be implemented for other autosomal recessive diseases in our population. PMID:21567908

  2. 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

  3. Segregative clustering of Lo and Ld membrane microdomains induced by local pH gradients in GM1-containing giant vesicles: a lipid model for cellular polarization.

    PubMed

    Staneva, Galya; Puff, Nicolas; Seigneuret, Michel; Conjeaud, Hlne; Angelova, Miglena I

    2012-11-27

    Several cell polarization processes are coupled to local pH gradients at the membrane surface. We have investigated the involvement of a lipid-mediated effect in such coupling. The influence of lateral pH gradients along the membrane surface on lipid microdomain dynamics in giant unilamellar vesicles containing phosphatidylcholine, sphingomyelin, cholesterol, and the ganglioside GM1 was studied. Lo/Ld phase separation was generated by photosensitization. A lateral pH gradient was established along the external membrane surface by acid local microinjection. The gradient promotes the segregation of microdomains: Lo domains within an Ld phase move toward the higher pH side, whereas Ld domains within an Lo phase move toward the lower pH side. This results in a polarization of the vesicle membrane into Lo and Ld phases poles in the axis of the proton source. A secondary effect is inward tubulation in the Ld phase. None of these processes occurs without GM1 or with the analog asialo-GM1. These are therefore related to the acidic character of the GM1 headgroup. LAURDAN fluorescence experiments on large unilamellar vesicles indicated that, with GM1, an increase in lipid packing occurs with decreasing pH, attributed to the lowering of repulsion between GM1 molecules. Packing increase is much higher for Ld phase vesicles than for Lo phase vesicles. It is proposed that the driving forces for domain vectorial segregative clustering and vesicle polarization are related to such differences in packing variations with pH decrease between the Lo and Ld phases. Such pH-driven domain clustering might play a role in cellular membrane polarization processes in which local lateral pH gradients are known to be important, such as migrating cells and epithelial cells. PMID:23121205

  4. Alpha-fucosidase-ganglioside interactions. Action of alpha-L-fucosidase from the hepatopancreas of Octopus vulgaris on a fucose-containing ganglioside (Fuc-GM1).

    PubMed

    Masserini, M; Giuliani, A; Venerando, B; Fiorilli, A; D'Aniello, A; Tettamanti, G

    1985-08-01

    alpha-L-Fucosidase, prepared in highly purified form (Mr 70 000-74 000) from Octopus hepatopancreas, was able to hydrolyse a fucose-containing ganglioside, namely Fuc-GM1 (II3NeuAc,IV2Fuc-GgOse4-Cer). The enzyme showed an irregular kinetic behaviour (v/[S] and v/[E] relationships following sigmoidal curves) when working on micellar Fuc-GM1 (Mr of the micelle 500 000), but obeyed regular hyperbolic kinetics when acting on low-Mr substances. It was observed that, on incubation with micellar Fuc-GM1 under the conditions used for the enzyme assay, Octopus alpha-L-fucosidase produced a ganglioside-enzyme complex that was catalytically inactive. This complex had an Mr exceeding 500 000 and a ganglioside/protein ratio of 4:1 (w/w), which is consistent with a stoichiometric combination of one ganglioside micelle with two enzyme molecules. Inactivation of alpha-L-fucosidase by formation of the corresponding complexes was also obtained with micellar gangliosides GM1 (II3NeuAc-GgOse4-Cer), GD1a (II3NeuAc,IV3NeuAc-GgOse4-Cer) and GT1b [II3(NeuAc)2,IV3-NeuAc-GgOse4-Cer], which are not substrates for the enzyme, indicating that the ganglioside micelles per se act as enzyme inhibitors. However, alpha-L-fucosidase easily forms a Fuc-GM1-alpha-L-fucosidase complex, displaying regular Michaelis-Menten kinetics. Therefore the anomalous behaviour exhibited by alpha-L-fucosidase on micellar Fuc-GM1 is likely due to formation of the complex, which separates the fucosyl linkage from the active site of the complexed enzyme, but makes it available to the enzyme in the free form. PMID:4052012

  5. Novel GM1 ganglioside-like peptide mimics prevent the association of cholera toxin to human intestinal epithelial cells in vitro.

    PubMed

    Yu, Robert K; Usuki, Seigo; Itokazu, Yutaka; Wu, Han-Chung

    2016-01-01

    Cholera is an acute diarrheal disease caused by infection in the gastrointestinal tract by the gram-negative bacterium, Vibrio cholerae, and is a serious public health threat worldwide. There has not been any effective treatment for this infectious disease. Cholera toxin (CT), which is secreted by V. cholerae, can enter host cells by binding to GM1, a monosialoganglioside widely distributed on the plasma membrane surface of various animal epithelial cells. The present study was undertaken to generate peptides that are conformationally similar to the carbohydrate epitope of GM1 for use in the treatment of cholera and related bacterial infection. For this purpose, we used cholera toxin B (CTB) subunit to select CTB-binding peptides that structurally mimic GM1 from a dodecamer phage-display library. Six GM1-replica peptides were selected by biopanning based on CTB recognition. Five of the six peptides showed inhibitory activity for GM1 binding to CTB. To test the potential of employing the peptide mimics for intervening with the bacterial infection, those peptides were examined for their binding capacity, functional inhibitory activity and in vitro effects using a human intestinal epithelial cell line, Caco-2 cells. One of the peptides, P3 (IPQVWRDWFKLP), was most effective in inhibiting cellular uptake of CTB and suppressing CT-stimulated cyclic adenosine monophosphate production in the cells. Our results thus provide convincing evidence that GM1-replica peptides could serve as novel agents to block CTB binding on epithelial cells and prevent the ensuing physiological effects of CT. PMID:26405107

  6. 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

  7. 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.

  8. 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

  9. [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

  10. Simulation of protein-sugar interactions: a computational model of the complex between ganglioside GM1 and the heat-labile enterotoxin of Escherichia coli.

    PubMed

    Bernardi, A; Raimondi, L; Zuccotto, F

    1997-06-01

    The cholera toxin from Vibrio cholerae (CT) and the 80% homologous heat-labile toxin of Escherichia coli (LT) are two well-known cases of sugar-binding proteins. The GM1:toxin complexes were chosen as test cases for the elaboration of a computational approach to the modeling of protein-saccharide interactions. The reliability of the method was evaluated on the LT:lactose complex. A model of this complex was built by performing a MC/EM conformational search of the sugar moiety within the binding pocket of LT, using the AMBER* force field and the GB/SA solvation model. The results are a reasonable reproduction of the reported X-ray structure of the complex. The same protocol was then applied to the LT:GM1 complex. The calculations were performed on a substructure that includes the protein shell within 5 A from GM1, three water molecules solvating Glu-51 carboxylate, and two water molecules at crystallographic sites 2 and 3. A satisfactory agreement was found with the recently published X-ray structure of the CT:GM1 complex. All the relevant interactions between the sugar and the residues involved in binding are well reproduced by the calculations. These results suggest that the substructure here identified can be taken as a realistic representation of the toxin binding surface and that the method presented in this paper can be used as a predictive tool in designing artificial LT (CT) binders and thus potential anticholera drugs. PMID:9191962

  11. The possible mechanism of Parkinson's disease progressive damage and the preventive effect of GM1 in the rat model induced by 6-hydroxydopamine.

    PubMed

    Xu, Renshi; Zhou, Yiyi; Fang, Xin; Lu, Yi; Li, Jiao; Zhang, Jie; Deng, Xia; Li, Shujuan

    2014-12-10

    The progressive pathogenesis and prevention of Parkinson's disease (PD) remains unknown at present. Therefore, the present study aimed to investigate the possible progressive pathogenesis and prevention of PD. Our study investigated the content of glutamate, mitochondria calcium, calmodulin, malonaldehyde and trace elements in striatum, cerebral cortex and hippocampus tissues; and the expression of bcl-2, bax and neuronal nitric oxide synthase (nNOS) in substantia nigra and striatum; and the change of apomorphine induced rotation behavior; and the treatmental effect of monosialotetrahexosylganglioside (GM1) intraperitoneal administration for 14 days in a PD rat model induced by 6-hydroxydopamine. The results revealed that the content of glutamate significantly decreased, and that of mitochondria calcium, calmodulin, malonaldehyde and ferrum significantly increased in striatum, cerebral cortex and hippocampus tissues; the content of magnesium significantly decreased, and that of cuprum and zinc significantly increased in cerebral cortex; the expression of bcl-2 significantly decreased, and that of bax and nNOS significantly increased in substantia nigra and striatum in PD rat. GM1 can partially improve the apomorphine induced rotation behavior and changes of glutamate, mitochondria calcium, calmodulin content in striatum of PD rat. Data suggested that dysfunction of excitatory amino acids neurotransmitter, calcium homeostasis disorder, abnormal metabolism of oxygen free radicals, abnormal trace elements distribution and/or deposition and excessive apoptosis participated in the progressive process of PD, and that GM1 could partially prevent the progressive damage. PMID:25285892

  12. GM1 and GD1a gangliosides modulate toxic and inflammatory effects of E. coli lipopolysaccharide by preventing TLR4 translocation into lipid rafts.

    PubMed

    Nikolaeva, Svetlana; Bayunova, Lubov; Sokolova, Tatyana; Vlasova, Yulia; Bachteeva, Vera; Avrova, Natalia; Parnova, Rimma

    2015-03-01

    Exogenous gangliosides are known to inhibit the effects of Escherichia coli lipopolysaccharide (LPS) in different cells exhibiting anti-inflammatory and immunosuppressive activities. The mechanisms underlying ganglioside action are not fully understood. Because LPS recognition and receptor complex formation occur in lipid rafts, and gangliosides play a key role in their maintenance, we hypothesize that protective effects of exogenous gangliosides would depend on inhibition of LPS signaling via prevention of TLR4 translocation into lipid rafts. The effect of GM1 and GD1a gangliosides on LPS-induced toxic and inflammatory reactions in PC12 cells, and in epithelial cells isolated from the frog urinary bladder, was studied. In PC12 cells, GD1a and GM1 significantly reduced the effect of LPS on the decrease of cell survival and on stimulation of reactive oxygen species production. In epithelial cells, gangliosides decreased LPS-stimulated iNOS expression, NO, and PGE2 production. Subcellular fractionation, in combination with immunoblotting, showed that pretreatment of cells with GM1, GD1a, or methyl-?-cyclodextrin, completely eliminated the effect of LPS on translocation of TLR4 into lipid rafts. The results are consistent with the hypothesis that ganglioside-induced prevention of TLR4 translocation into lipid rafts could be a mechanism of protection against LPS in various cells. PMID:25499607

  13. 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

  14. Functional interplay between ganglioside GM1 and cross-linking galectin-1 induces axon-like neuritogenesis via integrin-based signaling and TRPC5-dependent Ca(2+) influx.

    PubMed

    Wu, Gusheng; Lu, Zi-Hua; Andr, Sabine; Gabius, Hans-Joachim; Ledeen, Robert W

    2016-02-01

    Axon-like neuritogenesis in neuroblastoma (NG108-15) cells and primary cerebellar granular neurons is furthered by the presence of ganglioside GM1. We describe here that galectin-1 (Gal-1), a homobivalent endogenous lectin, is an effector by cross-linking the ganglioside and its associated glycoprotein ?5 ?1 -integrin. The thereby triggered signaling cascade involves autophosphorylation of focal adhesion kinase and activation of phospholipase C? and phosphoinositide-3 kinase. This leads to a transient increase in the intracellular Ca(2+) concentration by opening of TRPC5 channels, which belong to the signal transduction-gated cation channels. Controls with GM1-defective cells (NG-CR72 and neurons from ganglio-series KO mice) were retarded in axonal growth, underscoring the relevance of GM1 as functional counterreceptor for Gal-1. The lectin's presence was detected in the NG108-15 cells, suggesting an autocrine mechanism of action, and in astrocytes insitu. Gal-1, as cross-linking lectin, can thus translate metabolic conversion of ganglioside GD1a to GM1 by neuraminidase action into axon growth. Galectin-1 (Gal-1) was shown an effector of axonogenesis in cerebellar granule neurons (CGNs) and NG108-15 cells by cross-linking GM1 ganglioside and its associated glycoprotein ?5 ?1 -integrin. The resulting signaling led to a transient increase in intracellular Ca(2+) by opening TRPC5 channels. CGNs deficient in GM1 showed retarded axonogenesis, underscoring the relevance of GM1 as functional counterreceptor for Gal-1 in this process. This Gal-1/GM1-induced signaling was manifest only at the earliest, initiating stage of axon development. PMID:26526326

  15. 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

  16. 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

  17. Carbohydrate-to-carbohydrate interactions between ?2,3-linked sialic acids on ?2 integrin subunits and asialo-GM1 underlie the bone metastatic behaviour of LNCAP-derivative C4-2B prostate cancer cells.

    PubMed

    Van Slambrouck, Sverine; Groux-Degroote, Sophie; Krzewinski-Recchi, Marie-Ange; Cazet, Aurlie; Delannoy, Philippe; Steelant, Wim F A

    2014-01-01

    Complex interplays among proteins, lipids and carbohydrates can alter the phenotype and are suggested to have a crucial role in tumour metastasis. Our previous studies indicated that a complex of the GSLs (glycosphingolipids), AsGM1 (asialo-GM1), which lacks ?2,3-linked sialic acid, and ?2?1 integrin receptors is responsible for the metastatic behaviour of C4-2B prostate cancer cells. Herein, we identified and addressed the functional significance of changes in sialylation during prostate cancer progression. We observed an increase in ?2,3-linked sialic acid residues on ?2 subunits of ?2?1 integrin receptors, correlating with increased gene expression of ?2,3-STs (sialyltransferases), particularly ST3GAL3. Cell surface ?2,3-sialylation of ?2 subunits was required for the integrin ?2?1-dependent cell adhesion to collagen type I and the same ?2,3-linked sialic acid residues on the integrin receptor were responsible for the interaction with the carbohydrate moiety of AsGM1, explaining the complex formation between AsGM1 and ?2?1 integrin receptors. These results provide novel insights into the role of sialic acids in the organization and function of important membrane components in invasion and metastatic processes. PMID:25137483

  18. 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...

  19. 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.

  20. X-Ray Reflectivity and Grazing Incidence Diffraction Studies of Interaction between Human Adhesion/Growth-Regulatory Galectin-1 and DPPE-GM1 Lipid Monolayer at an Air/Water Interface.

    PubMed

    Majewski, J; Andr, S; Jones, E; Chi, E; Gabius, H-J

    2015-07-01

    The specific interaction of ganglioside GM1 with the homodimeric (prototype) endogenous lectin galectin-1 triggers growth regulation in tumor and activated effector T cells. This proven biorelevance directed interest to studying association of the lectin to a model surface, i.e. a 1,2-dihexadecanoyl-sn-glycero-3-phosphoethanolamine/ganglioside GM1 (80 : 20mol%) monolayer, at a bioeffective concentration. Surface expansion by the lectin insertion was detected at a surface pressure of 20 mN/m. On combining the methods of grazing incidence X-ray diffraction and X-ray reflectivity, a transient decrease in lipid-ordered phase of the monolayer was observed. The measured electron density distribution indicated that galectin-1 is oriented with its long axis in the surface plane, ideal for cis-crosslinking. The data reveal a conspicuous difference to the way the pentameric lectin part of the cholera toxin, another GM1-specific lectin, is bound to the monolayer. They also encourage further efforts to monitor effects of structurally different members of the galectin family such as the functionally antagonistic chimera-type galectin-3. PMID:26542007

  1. Therapeutic Potential of Intracerebroventricular Replacement of Modified Human ?-Hexosaminidase B for GM2 Gangliosidosis

    PubMed Central

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

    2011-01-01

    To develop a novel enzyme replacement therapy for neurodegenerative Tay-Sachs disease (TSD) and Sandhoff disease (SD), which are caused by deficiency of ?-hexosaminidase (Hex) A, we designed a genetically engineered HEXB encoding the chimeric human ?-subunit containing partial amino acid sequence of the ?-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

  2. 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

  3. GM2 gangliosidosis B1 variant: biochemical and molecular characterization of hexosaminidase A.

    PubMed

    Peleg, L; Meltzer, F; Karpati, M; Goldman, B

    1995-04-01

    The biochemical properties of hexosaminidase A (HexA) and the coding sequence of the alpha-subunit were examined in a patient of Syrian ancestry with the B1 form of Tay-Sachs disease (TSD). The biochemical characteristics of the variant HexA suggest that both active sites are affected by the mutation(s). Kinetic studies with the beta-subunit specific substrate, 4-methylumbelliferyl-beta-D-N-acetylglucosamine (MUG), revealed a significant difference between the Km values. of normal and variant HexA, while no difference was found when the sulfated substrate MUG-6-sulfate (MUGS), which is specific for the alpha-subunit active site, was used. The Vmax values for both substrates were significantly lower in extracts from B1 variant cells than in control extracts, implying a reduced enzyme level in the variant cells. A noncompetitive inhibitor of the reaction with MUGS, N-acetylglucosamine (NAG), induced a significant inhibition (30%) in the mutant cells only. When MUG was used as substrate, variant HexA was found to be more heat stable (T50 = 170 min) than normal HexA (T50 = 65 min). Furthermore, the mutant cell preparation differed from control in the relation between Hex thermosensitivity and protein concentration in the reaction. Two new mutations were identified in exon 5 of the HexA gene: a C496 to G transversion, which produced an Arg166 -->Gly alteration and a deletion of C498 which generated a shift in the reading frame. The patient was a heterozygote for both mutations even though her parents are first cousins. There is no evidence as yet which of these mutations accounts for the B1 phenotype. PMID:8581357

  4. Chaperone Therapy for Neuronopathic Lysosomal Diseases: Competitive Inhibitors as Chemical Chaperones for Enhancement of Mutant Enzyme Activities

    PubMed Central

    Suzuki, Yoshiyuki; Ogawa, Seiichiro; Sakakibara, Yasubumi

    2009-01-01

    Chaperone therapy is a newly developed molecular approach to lysosomal diseases, a group of human genetic diseases causing severe brain damage. We found two valienamine derivatives, N-octyl-4-epi-?-valienamine (NOEV) and N-octyl-?-valienamine (NOV), as promising therapeutic agents for human ?-galactosidase deficiency disorders (mainly GM1-gangliosidosis) and ?-glucosidase deficiency disorders (Gaucher disease), respectively. We briefly reviewed the historical background of research in carbasugar glycosidase inhibitors. Originally NOEV and NOV had been discovered as competitive inhibitors, and then their paradoxical bioactivities as chaperones were confirmed in cultured fibroblasts from patients with these disorders. Subsequently GM1-gangliosidosis model mice were developed and useful for experimental studies. Orally administered NOEV entered the brain through the blood-brain barrier, enhanced ?-galactosidase activity, reduced substrate storage, and improved neurological deterioration clinically. Furthermore, we executed computational analysis for prediction of molecular interactions between ?-galactosidase and NOEV. Some preliminary results of computational analysis of molecular interaction mechanism are presented in this article. NOV also showed the chaperone effect toward several ?-glucosidase gene mutations in Gaucher disease. We hope chaperone therapy will become available for some patients with GM1-gangliosidosis, Gaucher disease, and potentially other lysosomal storage diseases with central nervous system involvement. PMID:19812739

  5. Biomarkers of Central Nervous System Inflammation in Infantile and Juvenile Gangliosidoses

    PubMed Central

    Utz, Jeanine R.; Crutcher, Thomas; Schneider, Joseph; Sorgen, Patrick; Whitley, Chester B.

    2015-01-01

    Background The gangliosidoses (Tay-Sachs disease, Sandhoff disease and GM1-gangliosidosis) are progressive neurodegenerative diseases caused by lysosomal enzyme activity deficiencies and consequent accumulation of gangliosides in the central nervous system (CNS). The infantile forms are distinguished from the juvenile forms by age of onset, rate of disease progression and age of death. There are no approved treatments for the gangliosidoses. In search of potential biomarkers of disease, we quantified 188 analytes in CSF and serum from living human patients with longitudinal (serial) measurements. Notably, several associated with inflammation were elevated in the CSF of infantile gangliosidosis patients, and less so in more slowly progressing forms of juvenile gangliosidosis, but not in MPS disease. Thirteen CSF and two serum biomarker candidates were identified. Five candidate biomarkers were distinguished by persistent elevation in the CSF of patients with the severe infantile phenotype: ENA-78, MCP-1, MIP-1?, MIP-1?, TNFR2. Correspondence of abnormal elevation with other variables of disease --- i.e., severity of clinical phenotype, differentiation from changes in serum, and lack of abnormality in other neurodegenerative lysosomal diseases ---identifies these analytes as biomarkers of neuropathology specific to the gangliosidosis diseases. PMID:25557439

  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 ?-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, ?-domain 1 and ?-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. 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

  8. 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 ?-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 ?-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

  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. Diagnostic Approach to Childhood-onset Cerebellar Atrophy: A 10-Year Retrospective Study of 300 Patients

    PubMed Central

    Al-Maawali, Almundher; Blaser, Susan; Yoon, Grace

    2013-01-01

    Hereditary ataxias associated with cerebellar atrophy are a heterogeneous group of disorders. Selection of appropriate clinical and genetic tests for patients with cerebellar atrophy poses a diagnostic challenge. Neuroimaging is a crucial initial investigation in the diagnostic evaluation of ataxia in childhood, and the presence of cerebellar atrophy helps guide further investigations. We performed a detailed review of 300 patients with confirmed cerebellar atrophy on magnetic resonance imaging over a 10-year period. A diagnosis was established in 47% of patients: Mitochondrial disorders were most common, followed by the neuronal ceroid lipofuscinoses, ataxia telangectasia, and late GM2-gangliosidosis. We review the common causes of cerebellar atrophy in childhood and propose a diagnostic approach based on correlating specific neuroimaging patterns with clinical and genetic diagnoses. PMID:22764178

  11. Prenatal and postnatal studies of a late infantile GM2 gangliosidosis in a family of Syrian origin: a possible B1 variant.

    PubMed

    Shukry, A; Goldman, B; Shihab, S; Peleg, L

    1993-10-01

    We describe late infantile Tay-Sachs disease with high residual hexosaminidase A activity in two siblings of a Syrian Druze family. The patients' leukocytes had 26% of normal hexosaminidase A activity when tested with the conventional fluorogenic substrate 4-methyl-umbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside (4-MUG) and only about 10% when assayed with the sulfated substrate, 4-methyl-umbelliferal- beta-N-acetyl-glucosamine-6-sulfate (4-MUGS). According to the standard procedure of the heterozygote screening program (employing 4-MUG and heat inactivation), the parents were not diagnosed as an at-risk couple since the father was classified as a noncarrier. However, both parents' levels were clearly within the carrier range on the basis of 4-MUGS. The unique catalytic characteristics of the patients' enzyme forward the assumption that the affected sibs are B1 variants. The parents' enzymatic levels, together with their known consanguinity, might indicate that these patients are homozygotes for the rare mutation and not genetic compounds as has been documented for most of the infantile B1 variants. To the best of our knowledge this is the first reported case of B1 variant in a child of that extraction. PMID:8244659

  12. 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

  13. Adult type neuronal storage disease with neuraminidase deficiency.

    PubMed

    Miyatake, T; Atsumi, T; Obayashi, T; Mizuno, Y; Ando, S; Ariga, T; Matsui-Nakamura, K; Yamada, T

    1979-09-01

    We describe a patient with adult-onset neuronal storage disease characterized by myoclonus, cerebellar ataxia, convulsive seizures, cherry-red spots, skeletal dysplasia, mild gargoyle features, inguinal hernia, and angiokeratoma. Cytoplasmic inclusions consistent with lysosomal storage disease were demonstrated in neurons of the autonomic nervous system. Accumulation of GM3 and GM2 gangliosides was found in sympathetic ganglia but a catabolic disturbance of these gangliosides was ruled out by normal levels of GM3 ganglioside sialidase and N-acetyl-beta-hexosaminidase A activities. beta-Galactosidase activity was decreased in leukocytes and fibroblasts, but not in serum. GM1 gangliosidosis was ruled out by lipid analyses, and mucopolysaccharidosis by normal excretion of mucopolysaccharide in urine. Sialyl oligosaccharides were increased in urine and alpha-neuraminidase was deficient in fibroblasts. This disorder is considered to be an inherited metabolic disorder of sialyl glycoproteins and oligosaccharides due to deficiency of an alpha-neuraminidase. PMID:534422

  14. Synthesis of 1,5-dideoxy-1,5-iminoribitol C-glycosides through a nitrone-olefin cycloaddition domino strategy: identification of pharmacological chaperones of mutant human lysosomal ?-galactosidase.

    PubMed

    Siriwardena, Aloysius; Sonawane, Dhiraj P; Bande, Omprakash P; Markad, Pramod R; Yonekawa, Sayuri; Tropak, Michael B; Ghosh, Sougata; Chopade, Balu A; Mahuran, Don J; Dhavale, Dilip D

    2014-05-16

    We report herein a newly developed domino reaction that facilitates the synthesis of new 1,5-dideoxy-1,5-iminoribitol iminosugar C-glycosides 7a-e and 8. The key intermediate in this approach is a six-membered cyclic sugar nitrone that is generated in situ and trapped by an alkene dipolarophile via a [2 + 3] cycloaddition reaction to give the corresponding isooxazolidines 10a-e in a "one-pot" protocol. The iminoribitol C-glycosides 7a-e and 8 were found to be modest ?-galactosidase (bGal) inhibitors. However, compounds 7c and 7e showed "pharmacological chaperone" activity for mutant lysosomal bGal activity and facilitated its recovery in GM1 gangliosidosis patient fibroblasts by 2-6-fold. PMID:24735108

  15. 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

  16. 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

  17. 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

  18. 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.

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

    MedlinePLUS

    ... for making a protein called the GM2 ganglioside activator. This protein is required for the normal function ... cord. Beta-hexosaminidase A and the GM2 ganglioside activator protein work together in lysosomes, which are structures ...

  20. 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

  1. 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

  2. Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

    PubMed Central

    Davidson, Cristin D.; Ali, Nafeeza F.; Micsenyi, Matthew C.; Stephney, Gloria; Renault, Sophie; Dobrenis, Kostantin; Ory, Daniel S.; Vanier, Marie T.; Walkley, Steven U.

    2009-01-01

    Background Niemann-Pick type C (NPC) disease is a fatal neurodegenerative disorder caused most commonly by a defect in the NPC1 protein and characterized by widespread intracellular accumulation of unesterified cholesterol and glycosphingolipids (GSLs). While current treatment therapies are limited, a few drugs tested in Npc1?/? mice have shown partial benefit. During a combination treatment trial using two such compounds, N-butyldeoxynojirimycin (NB-DNJ) and allopregnanolone, we noted increased lifespan for Npc1?/? mice receiving only 2-hydroxypropyl-?-cyclodextrin (CD), the vehicle for allopregnanolone. This finding suggested that administration of CD alone, but with greater frequency, might provide additional benefit. Methodology/Principal Findings Administration of CD to Npc1?/? mice beginning at either P7 or P21 and continuing every other day delayed clinical onset, reduced intraneuronal cholesterol and GSL storage as well as free sphingosine accumulation, reduced markers of neurodegeneration, and led to longer survival than any previous treatment regime. We reasoned that other lysosomal diseases characterized by cholesterol and GSL accumulation, including NPC disease due to NPC2 deficiency, GM1 gangliosidosis and mucopolysaccharidosis (MPS) type IIIA, might likewise benefit from CD treatment. Treated Npc2?/? mice showed benefits similar to NPC1 disease, however, mice with GM1 gangliosidosis or MPS IIIA failed to show reduction in storage. Conclusions/Significance Treatment with CD delayed clinical disease onset, reduced intraneuronal storage and secondary markers of neurodegeneration, and significantly increased lifespan of both Npc1?/? and Npc2?/? mice. In contrast, CD failed to ameliorate cholesterol or glycosphingolipid storage in GM1 gangliosidosis and MPS IIIA disease. Understanding the mechanism(s) by which CD leads to reduced neuronal storage may provide important new opportunities for treatment of NPC and related neurodegenerative diseases characterized by cholesterol dyshomeostasis. PMID:19750228

  3. Normalizing glycosphingolipids restores function in CD4+ T cells from lupus patients.

    PubMed

    McDonald, Georgia; Deepak, Shantal; Miguel, Laura; Hall, Cleo J; Isenberg, David A; Magee, Anthony I; Butters, Terry; Jury, Elizabeth C

    2014-02-01

    Patients with the autoimmune rheumatic disease systemic lupus erythematosus (SLE) have multiple defects in lymphocyte signaling and function that contribute to disease pathogenesis. Such defects could be attributed to alterations in metabolic processes, including abnormal control of lipid biosynthesis pathways. Here, we reveal that CD4+ T cells from SLE patients displayed an altered profile of lipid raft-associated glycosphingolipids (GSLs) compared with that of healthy controls. In particular, lactosylceramide, globotriaosylceramide (Gb3), and monosialotetrahexosylganglioside (GM1) levels were markedly increased. Elevated GSLs in SLE patients were associated with increased expression of liver X receptor ? (LXR?), a nuclear receptor that controls cellular lipid metabolism and trafficking and influences acquired immune responses. Stimulation of CD4+ T cells isolated from healthy donors with synthetic and endogenous LXR agonists promoted GSL expression, which was blocked by an LXR antagonist. Increased GSL expression in CD4+ T cells was associated with intracellular accumulation and accelerated trafficking of GSL, reminiscent of cells from patients with glycolipid storage diseases. Inhibition of GSL biosynthesis in vitro with a clinically approved inhibitor (N-butyldeoxynojirimycin) normalized GSL metabolism, corrected CD4+ T cell signaling and functional defects, and decreased anti-dsDNA antibody production by autologous B cells in SLE patients. Our data demonstrate that lipid metabolism defects contribute to SLE pathogenesis and suggest that targeting GSL biosynthesis restores T cell function in SLE. PMID:24463447

  4. 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 ?-galactosidase A, ? glucosidase, ?-L-iduronidase, arylsulfatase B, ?-galactosidase, ?-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

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. Patient Advocacy

    Cancer.gov

    CPTAC recognizes the important role that patients, patient advocates, and other members outside of the traditional science community play in advancing cancer research, and is actively involved in dialogue with such communities. To this end the consortium

  11. 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

  12. BEN Patient

    USGS Multimedia Gallery

    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 ...

  13. 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

  14. 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

  15. Carrier rates of four single-gene disorders in Croatian Bayash Roma.

    PubMed

    Barei?, Ana; Peri?i? Salihovi?, Marijana

    2014-02-01

    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 Me?imurje 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

  16. 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

  17. Patient Roadmap

    MedlinePLUS

    ... support. Please note: this “Roadmap” outlines a general process and is designed to apply to the broadest array of tinnitus cases. While most patients would be well served following the progressive approach below, each patient’s ...

  18. Patient Rights

    MedlinePLUS

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

  19. 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

  20. 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.

  1. Patient Corner

    Cancer.gov

    Biospecimens are biological materials from people (such as tissue, blood, plasma, and urine) that can be used for diagnosis and basic research. When cancer patients undergo procedures such as a blood draw, biopsy or surgery in which a specimen is removed for diagnostic or therapeutic purposes, it is often possible for a small amount of residual specimen to be stored and later used for research.

  2. 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

  3. 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

  4. 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 Crohns 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

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. [What patients think of patient education].

    PubMed

    Giroux, Valrie

    2013-10-01

    What patients think of therapeutic education. Therapeutic education for patients has proven beneficial in the management of many chronic diseases. At the Marie-Madeleine hospital centre in Forbach, France, where a therapeutic education unit has been in place since 2006, a satisfaction survey undertaken in a group of patients with heart failure shows areas for improvement. PMID:24288891

  11. 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

  12. National Patient Safety Foundation

    MedlinePLUS

    ... Now Store Donate Help Contact Home Creating a world where patients and those who care for them ... Patient Blood Management Reducing Diagnostic Error Health Info Technology Publications Ask Me 3 For Patients and Families ...

  13. 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)

  14. 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

  15. Patient Education on Pain

    MedlinePLUS Videos and Cool Tools

    ... People with Pain Press Room Position Statements Patient Education on Pain AAPM Past President, Perry G. Fine, ... Member Center Patient Center Research Advocacy Practice Management Education Annual Meeting Contact Us Privacy Policy Sitemap Close ...

  16. Patient Emergency Grants

    MedlinePLUS

    ... Street, Suite G Freehold, NJ 07728 Patient Emergency Grants The Kidney & Urology Foundation of America, now in ... Kidney & Urology Foundation of Americas (KUFA) PATIENT EMERGENCY GRANT PROGRAM (PEG) provides financial assistance to End Stage ...

  17. 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

  18. NORD's Patient Assistance Programs

    MedlinePLUS

    ... Assistance Programs Patient Workshops Patient Organization Programs Rare Action Network™ Event Sponsorship Rare Impact Awards Summit Rare Disease Day Education & Research Programs Physician Guides Rare Disease Reports Natural Histories ...

  19. 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

  20. Managing patients with encephalitis.

    PubMed

    Matata, Claire; Easton, Ava; Michael, Benedict; Evans, Becky; Ward, Deborah; Solomon, Tom; Kneen, Rachel

    2015-11-11

    This article provides an overview of encephalitis and addresses its diagnosis, some of the common presenting signs and symptoms, and the different aspects of nursing care required for these patients. In particular, it addresses how to explain encephalitis to the patient's relatives, the rehabilitation needs of these patients, and important aspects of discharge planning. Tests that are necessary for diagnosis in patients with suspected encephalitis and the importance of these are explained. PMID:26554998

  1. [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

  2. 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

  3. Patients with chronic pain.

    PubMed

    Salama-Hanna, Joseph; Chen, Grace

    2013-11-01

    Preoperative evaluation of patients with chronic pain is important because it may lead to multidisciplinary preoperative treatment of patients' pain and a multimodal analgesia plan for effective pain control. Preoperative multidisciplinary management of chronic pain and comorbid conditions, such as depression, anxiety, deconditioning, and opioid tolerance, can improve patient satisfaction and surgical recovery. Multimodal analgesia using pharmacologic and nonpharmacologic strategies shifts the burden of analgesia away from simply increasing opioid dosing. In more complicated chronic pain patients, multidisciplinary treatment, including pain psychology, physical therapy, judicious medication management, and minimally invasive interventions by pain specialists, can improve patients' satisfaction and surgical outcome. PMID:24182727

  4. 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

  5. The problematic asthma patient

    PubMed Central

    Kui, Swee Leng; How, Choon How; Koh, Jansen

    2015-01-01

    Asthma is a reversible chronic inflammatory disorder of the airways that can be effectively controlled without causing any lifestyle limitation or burden on the quality of life of the majority of asthma patients. However, persistently uncontrolled asthma can be frustrating for both the patient and the managing physician. Patients who fail to respond to high-intensity asthma treatment fall into the category of problematic asthma, which is further subdivided into difficult asthma and severe refractory asthma. Establishing the correct diagnosis of asthma and addressing comorbidities, compliance, inhaler technique and environmental triggers are essential when dealing with problematic asthma patients. A systemic approach is also crucial in managing such patients. This is pertinent for general practitioners, as the majority of asthma patients are diagnosed and managed at the primary care level. PMID:26243972

  6. Guaranteeing patient satisfaction.

    PubMed

    Levin, R P

    1994-04-01

    Dental practice is about people and service. As the competition for patients increases, the successful practices will be the ones that focus increasingly on communication, relationship building and customer service. Quality care will be demonstrated through focusing on the total clinical and psychological aspects of patient satisfaction--and not just on technical parameters. Any practice can convert to a high level patient relations office if the commitment is truly there. These will be the leading practices of tomorrow. PMID:9520766

  7. 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)

  8. Patient-physician communication.

    PubMed

    Asnani, M R

    2009-09-01

    Extensive research has shown that no matter how knowledgeable the physician might be, if he/she is not able to open good communication channels with the patient, he/she may be of no help to the latter Despite this known fact and the fact that a patient-physician consultation is the most widely performed 'procedure' in a physician's professional lifetime, effective communication with the patient has been found to be sadly lacking. This review article seeks to discuss 'the what', 'the why' and 'the how' of doctor-patient communication. PMID:20099777

  9. Physicians as Patient Teachers

    PubMed Central

    Brunton, Stephen A.

    1984-01-01

    Physicians have a central role in educating patients and the public in the elements of personal health maintenance. To be an effective teacher, one must recognize the learning needs of each patient and use methods of information transfer that will result in comprehension and compliance. To bring about a change in life-style, one must also have an understanding of a patient's health beliefs and the determinants of human behavior. Using this information together with behavior modification strategies, physicians can forge an effective partnership with patients working toward the goal of optimum health. PMID:6395500

  10. Patient adherence in COPD

    PubMed Central

    Bourbeau, J; Bartlett, S J

    2015-01-01

    Patient adherence to treatment in chronic obstructive pulmonary disease (COPD) is essential to optimise disease management. As with other chronic diseases, poor adherence is common and results in increased rates of morbidity, healthcare expenditures, hospitalisations and possibly mortality, as well as unnecessary escalation of therapy and reduced quality of life. Examples include overuse, underuse, and alteration of schedule and doses of medication, continued smoking and lack of exercise. Adherence is affected by patients perception of their disease, type of treatment or medication, the quality of patient provider communication and the social environment. Patients are more likely to adhere to treatment when they believe it will improve disease management or control, or anticipate serious consequences related to non-adherence. Providers play a critical role in helping patients understand the nature of the disease, potential benefits of treatment, addressing concerns regarding potential adverse effects and events, and encouraging patients to develop self-management skills. For clinicians, it is important to explore patients beliefs and concerns about the safety and benefits of the treatment, as many patients harbour unspoken fears. Complex regimens and polytherapy also contribute to suboptimal adherence. This review addresses adherence related issues in COPD, assesses current efforts to improve adherence and highlights opportunities to improve adherence for both providers and patients. PMID:18728206

  11. 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

  12. 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

  13. [Polyneuropathy in elderly patients].

    PubMed

    Akhmedzhanova, L T; Barinov, A N

    2015-01-01

    In this article, the authors consider the characteristics of pathogenesis and clinical picture of peripheral nervous system lesions in elderly patients. Current approaches to pathogenetic and symptomatic treatment of polyneuropathy in elderly patients are analyzed. The authors suggest complex treatment approaches using antiepileptic drugs, group B vitamins, alpha-lipoic acid. PMID:26171485

  14. Mimivirus in Pneumonia Patients

    PubMed Central

    La Scola, Bernard; Marrie, Thomas J.; Auffray, Jean-Pierre

    2005-01-01

    Mimivirus, the largest virus known to date, is an amebal pathogen like Legionella spp. When mimivirus was used as an antigen in a migration inhibition factor assay, seroconversion was found in patients with both community- and hospital-acquired pneumonia. Mimivirus DNA was found in respiratory samples of patients with hospital-acquired pneumonia. PMID:15757563

  15. Mimivirus in pneumonia patients.

    PubMed

    La Scola, Bernard; Marrie, Thomas J; Auffray, Jean-Pierre; Raoult, Didier

    2005-03-01

    Mimivirus, the largest virus known to date, is an amebal pathogen-like Legionella sp. When Mimivirus was used as an antigen in a microimmunofluorescense assay, seroconversion was found in patients with both community- and hospital-acquired pneumonia. Mimivirus DNA was found in respiratory samples of a patient with hospital-acquired pneumonia. PMID:15757563

  16. 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…

  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. 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

  19. 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

  20. 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

  1. [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

  2. Strongyloidiasis in Transplant Patients

    PubMed Central

    Roxby, Alison C.; Gottlieb, Geoffrey S.; Limaye1, Ajit P.

    2010-01-01

    Strongyloides stercoralis is an intestinal nematode that can persist in the human host for decades after the initial infection and can progress to fulminant hyperinfection syndrome in immunocompromised hosts. We describe a patient who died of Strongyloides hyperinfection syndrome 2 months after orthotopic heart transplantation and discuss approaches to prevention, diagnosis, and treatment. Current practice guidelines recommend screening for and treatment of Strongyloides infection before transplantation, but physicians in the United States often miss opportunities to identify patients with chronic strongyloidiasis. Screening tests have limitations, and clinical suspicion remains an important component of the evaluation before transplantation. After immunocompromised patients develop hyperinfection syndrome, diagnosis is often delayed and mortality is high, so emphasis must be placed on screening and treatment before transplantation. We review current strategies for prevention, diagnosis, and treatment of chronic intestinal strongyloidiasis in patients who will undergo transplantation and discuss the clinical features and management of Strongyloides hyperinfection syndrome in transplant recipients. PMID:19807271

  3. 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 ...

  4. Patient satisfaction constructs.

    PubMed

    Rahman, Muhammad Sabbir; Osmangani, Aahad M

    2015-10-12

    Purpose - The purpose of this paper is to examine the five-factor structure of patients' satisfaction constructs toward private healthcare service providers. Design/methodology/approach - This research is a cross-sectional study. A questionnaire-based survey was conducted with previous and current Bangladeshi patients. Exploratory factor analysis was employed to extract the underlying constructs. Findings - Five underlying dimensions that play a significant role in structuring the satisfaction perceived by Bangladeshi private healthcare patients are identified in this study. Practical implications - The main contribution of this study is identifying the dimensions of satisfaction perceived by Bangladeshi patients regarding private healthcare service providers. Originality/value - Healthcare managers adopt the five identified underlying construct items in their business practices to improve their respective healthcare efficiency while ensuring overall customer satisfaction. PMID:26440486

  5. Patient's Bill of Rights

    MedlinePLUS

    ... on January 2, 2014. Families USA. The Affordable Care Act: Patients’ Bill of Rights and Other Protections. April 2011. Accessed at http://familiesusa2.org/assets/pdfs/health-reform/Patients-Bill-of-Rights.pdf on January 2, ...

  6. [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

  7. Appendicitis in mature patients.

    PubMed Central

    Burns, R P; Cochran, J L; Russell, W L; Bard, R M

    1985-01-01

    All patients greater than 50 years of age (N = 96) admitted with a pre- or postoperative diagnosis of acute appendicitis from 1971 to 1980 were reviewed. A comparative series of 91 patients aged 25 to 50 years was similarly reviewed. Noninflammatory diseases of the appendix and incidental appendectomies were excluded. Detailed study of symptoms, clinical presentation, laboratory evaluation, radiographic evaluation, concomitant diseases, hospital course, surgical findings, complications, and mortality were completed. Comparison of patients aged 25 to 50 to patients older than 50 years revealed a statistically significant increased incidence of perforation in the older group (p less than 0.0001). Sixty-five per cent of the older group showed greater incidence of perforation. Further analysis of this series yields the hypothesis that the increased incidence of perforation is related to a significant decrease in the frequency of classic presentation in the greater-than-50 age group, a significant decrease in frequency of correct admission diagnosis and a significant delay between admission and surgical procedure in the older group. A more rapid pathophysiologic progression of appendicitis with increasing age was noted. A much higher percentage of older patients was undiagnosed until the surgical procedure. In this group, there was a longer duration of symptoms, less frequent classic presentation, and decreased frequency of right lower quadrant guarding and tenderness as compared to patients with correct diagnosis prior to surgery. Complications were much more frequent in older patients and higher still in those with perforation. Analysis of findings by decade of life revealed an anticipated high incidence of perforated appendicitis in patients greater than 50, but also showed a continuation of the high incidence of perforation into the decade 40 to 50. There were three deaths in the entire study group (1.6%) all occurring in the older age group with postoperative sepsis. PMID:4004382

  8. Patient care in radiography

    SciTech Connect

    Ehrlich, R.A.; McCloskey, E.D.

    1989-01-01

    This book focuses on patient care procedures for radiographers. The authors focus on the role of the radiographer as a member of the health care team. The authors report on such topics as communication in patient care: safety, medico-legal considerations, transfer and positioning; physical needs; infection control; medication; CPR standards, acute situations; examination of the GI tract; contrast media; special imaging techniques and bedside radiography.

  9. [Jehovah's Witness patients].

    PubMed

    Yorozu, Tomoko

    2010-09-01

    There are still legal and ethical problems relating to the refusal of blood transfusion due to religious belief of Jehovah's Witness patients. Since general consensus about the anesthetic management of these patients has not been established, the "Guidelines for refusal of blood transfusion on religious grounds" was proposed by the joint committee including Japanese Society of Anesthesiologists in February 2008. The principle of the guideline is that if a patient, who refuses blood transfusion even at cost of life, is 18 years old or above and competent to understand the medical treatment and the adverse effects due to bloodless medicine, doctors may plan and complete bloodless surgery. The same policy is allowed if the patient is 15 years old or above and he/she as well as all the parental authorities refuse blood transfusion. In other cases, the policy of life-saving blood transfusion should be explained to the patients. If a bloodless surgery is planned, precise perioperative strategies to avoid blood loss have to be carried out with the cooperation of all the physicians who care the patient. Anesthesiologists should be carefully prepared for the possible law suits that might lead to unexpected outcomes. PMID:20857672

  10. The patient's view.

    PubMed

    Rachagan, S S; Sharon, K

    2003-03-01

    The medical practitioner has always had to juggle several roles. First and foremost, the doctor is a healer, a provider of curative services. Second, he is an examiner, an assessor of the patient's health status. Third, he is a researcher, always trying to push the boundaries of medical knowledge. Fourth, he is a rationer of services, he decides how best to apportion the limited resources at his disposal. Traditionally, the patient-doctor relationship has been largely exclusive in nature and the doctor would quite comfortably slip in and out of these roles, his focus centred on his patient's interests. In this era of large corporate health care providers, multi-billion-biotechnology industry, mammoth pharmaceutical companies, medical insurance schemes and international trade instruments, it has become increasingly difficult for the doctor to juggle these four roles. He is constantly subjected to conflicting demands. Patients' interests do not always come first anymore and patients are beginning to realise this. They no longer trust the medical profession unreservedly. There has been steady erosion of the patient-doctor relationship most clearly evidenced by the rising tide of litigation against doctors. There needs to be a reappraisal of these roles that the doctor plays. The conflicts must be recognised and addressed. Patients need to be informed and their interests must be protected if the doctor-patient relationship is to be restored. Medical malpractice suits are on the increase. The tort system as it exists is failing both doctors and patients. The question we must ask is what are patients looking for when they sue doctors? Most of the time they need compensation for the injuries suffered. Sometimes they are looking for accountability, they want the doctor to be punished in some way. Sometimes they merely want to air their grievances and know that they are heard. The current system more often than not takes too long to compensate, the process is a gamble and doctors who are clearly negligent quietly settle and are rarely censured. We need to revamp the existing system to allow for speedy and equitable compensation; true accountability; and articulation and auditing of standards of practice. PMID:14556356

  11. [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

  12. [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

  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. Doctor's Empathy Boosts Patient Satisfaction

    MedlinePLUS

    ... nih.gov/medlineplus/news/fullstory_157738.html Doctor's Empathy Boosts Patient Satisfaction Study finds wait time, appointment ... their hand surgeon. The results showed that doctor empathy accounted for 65 percent of patient satisfaction. Patient ...

  15. 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 ...

  16. 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

  17. Fatigue in cancer patients.

    PubMed Central

    Smets, E. M.; Garssen, B.; Schuster-Uitterhoeve, A. L.; de Haes, J. C.

    1993-01-01

    In this paper an overview is presented on what is currently known of fatigue in cancer. Fatigue is considered to be a multi-dimensional concept, that should be measured as such. However, fatigue has been assessed mostly by single items in general symptom checklists. The few specific instruments that have been used in cancer patient populations are discussed. The majority of cancer patients, about 70%, report feelings of fatigue during radio- or chemotherapy. Follow-up results show that, at least for some diagnoses, patients remain fatigued long after treatment has ended. Somatic and psychological mechanisms that have been proposed to explain fatigue are discussed. It is argued that the significance of the results obtained on fatigue as a symptom in cancer depends on comparison with other patient and non-patient populations. Also the occurrence of a response-shift has to be considered, leading to under reporting of fatigue. Finally, possible interventions to decrease feelings of fatigue are presented. PMID:8347475

  18. Biomarkers in hemodialysis patients.

    PubMed

    Hojs, Radovan; Bevc, Sebastjan; Ekart, Robert

    2012-01-01

    Patients with chronic kidney disease (CKD) are, compared to the general population, at higher risk of cardiovascular disease (CVD), including sudden death, coronary artery disease (CAD), congestive heart failure (HF), stroke, and peripheral artery disease. The presence of CVD is independently associated with kidney function decline. Renal insufficiency is a strong and independent predictor of mortality in patients with different CKD stages. The interplay of traditional and nontraditional risk factors is complex such that risk factor profiles are different in CKD patients. Seemingly, paradoxical associations between traditional risk factors and cardiovascular outcome complicate efforts to identify real cardiovascular etiology in these patients. Additional tools are often required to aid clinical assessment of cardiovascular risk. Recently, a number of cardiovascular biomarkers were identified as predictors of outcome in CVD. These may be used to guide early diagnosis and therapy for CVD or may predict outcome in CKD. This review focuses on the potential diagnostic and prognostic use of some important new biomarkers including brain natriuretic peptide (BNP), cardiac troponins (cTns), inflammatory markers, adhesion molecules, and asymmetric dimethylarginine (ADMA) in CKD as well as those patients with end-stage renal failure. PMID:22870586

  19. Perinatal Patient Safety Project

    PubMed Central

    Nunes, Julie; McFerran, Sharon

    2005-01-01

    The Perinatal Patient Safety Project (PPSP) was created as a systemic strategy for creating high-reliability perinatal units by preventing identified causes of perinatal events in the clinical setting. With developmental funding from a Garfield grant, implementation of the PPSP has been completed at four pilot sites in the Kaiser Permanente Northern California (KPNC) Region. Its success has resulted in implementation at all perinatal units in the KPNC Region as well as being promoted by National Risk Management for nationwide implementation. PPSP emphasizes structured communication, multidisciplinary rounds, a definition of fetal well-being, and practicing for emergencies. Steps taken to create high reliability perinatal care include improved communication, patient safety focus, and satisfaction among perinatal patients, providers, and staff. PMID:21660157

  20. [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

  1. Mental patients in prisons

    PubMed Central

    ARBOLEDA-FLREZ, 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

  2. Why Patients Choose Homeopathy

    PubMed Central

    Avina, Robert L.; Schneiderman, Lawrence J.

    1978-01-01

    Interviews with 100 homeopathic patients in the San Francisco Bay Area show that for the most part the patients are young, white and well-educated, and have white-collar jobs; most had previously tried mainstream medical care and found it unsatisfactory. Among the reasons for their dissatisfaction were instances of negative side effects from medication, lack of nutritional or preventive medical counseling, and lack of health education. Experiences with conventional physicians were almost evenly divided: nearly half of the subjects reported poor experiences, slightly fewer reported good experiences. Three quarters of the patients suffered from chronic illness and about half considered their progress to be good under homeopathic care. The majority were simultaneously involved in other nontraditional health care activities. PMID:664638

  3. 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

  4. 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

  5. Rhinoplasty in Latino Patients.

    PubMed

    Cobo, Roxana

    2016-01-01

    Rhinoplasty is the main facial plastic procedure performed in Latin America. Mestizo or Latino patients tend to have noses with thick skin, bulbous tips with poor projection, and flimsy osteocartilaginous underlying frameworks. A technique is presented in which structural grafts are used to strengthen support structures of the nose. A gradual approach is used to obtain tip definition, rotation, and projection using sutures and grafts. Simple techniques are used initially, progressing to more aggressive and less predictable ones in patients who require greater changes. The result should be noses that look more refined, with greater definition, but without looking bigger. PMID:26616711

  6. The dangerous patient.

    PubMed

    Lande, R G

    1989-07-01

    The dangerous patient requires proper management to ensure appropriate disposition and preclude injury. A safety-conscious public has propelled the issue into the courtroom, and a slowly evolving standard of care is emerging. In some states a legal duty to protect victims of violence exists. Understanding human aggression, the potentiating effects of the environment, and prior methods of coping, as well as assessing current behavioral controls and certain statistical correlates all aid management decisions. Stabilization of the dangerous patient begins with the interview and progresses to medication or restraints as the situation dictates. Once the acute crisis is resolved, attention is directed toward a more thorough review. PMID:2738552

  7. Improving patient cooperation.

    PubMed

    Carroll, Q B

    1979-01-01

    Patient anxiety and poor cooperation are more apt to be socio-psychological in origin rather than mental or physical. Therefore, it is important that the technology curriculum provide sufficient emphasis on behavioral psychology, motivation and control techniques, and communication skills. Studies show that if the patient and his family are adequately informed of what to expect when they go to the radiology department, through interviews, videotapes, etc., their anxieties decrease. Use of anxiety-reducing techniques can decrease uncooperative behavior, examination time, and misunderstandings that cause a poor professional image. PMID:472224

  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. 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)

  11. 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

  12. 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

  13. 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…

  14. 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

  15. 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

  16. 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

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

    PubMed

    Tourette-Turgis, Catherine

    2015-10-01

    The Patients' University, a pilot project at the Universit 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. Training Patient and Family Storytellers and Patient and Family Faculty

    PubMed Central

    Morrise, Lisa; Stevens, Katy Jo

    2013-01-01

    Narrative medicine has become a prominent method of developing more empathetic relationships between medical clinicians and patients, on the basis of a deeper understanding of the patient experience. Beyond its usefulness during clinical encounters, patient storytelling can inform processes and procedures in Advisory Councils, Committee Meetings, and Family as Faculty settings, leading to improved quality and safety in health care. Armed with a better understanding of the patient experience, clinicians and administrators can make decisions, hopefully in collaboration with patients, that will enrich the patient experience and increase satisfaction among patients, families, and staff. Patient and family storytelling is a key component of the collaboration that is ideal when an organization seeks to deliver patient- and family-centered care. Providing patients and families with training will make the narratives they share more powerful. Health care organizations will find that purposeful storytelling can be an invaluable aspect of a patient- and family-centered culture. Well-delivered storytelling will support quality- and safety-improvement efforts and contribute to improved patient satisfaction. This article provides instruction for teaching patients and families how to tell stories with purpose and offers advice about how to support patients, families, and clinicians participating in this effort. PMID:24355906

  19. [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

  20. 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

  1. [The XXI century patient].

    PubMed

    Jovell, A J

    2006-01-01

    We are experiencing a social change in health that is unprecedented in the history of humanity. Modern sociology attempts to explain this change using concepts of reflexive modernisation, liquid life or society, or the end of history. This social change results in a new model of patient who is better informed and has greater expectations with respect to healthcare and health. More information does not mean better understanding and more responsibility with respect to health. For this to occur, it is necessary to increase the social and individual conscience of people as health agents and as responsible users. The University of the Patients emerges as a project directed towards increasing the health and civic literacy of the population and from the need to adapt the health systems to the new needs generated by a new model of user. PMID:17308542

  2. Gastric Cancer in Young Patients

    PubMed Central

    Dhobi, Manzoor A.; Wani, Khursheed Alam; Parray, Fazl Qadir; Wani, Rouf A.; Peer, G. Q.; Abdullah, Safiya; Wani, Imtiyaz A.; Wani, Muneer A.; Shah, Mubashir A.; Thakur, Natasha

    2013-01-01

    Aim. The aim of this study was to see the clinical, pathological, and demographic profile of young patients with stomach carcinoma besides association with p53. Patients and Methods. Prospective study of young patients with stomach carcinoma from January 2005 to December 2009. A total of 50 patients with age less than 40 years were studied. Results. Male female ratio was 1?:?1.08 in young patients and 2.5?:?1 in older patients. A positive family history of stomach cancer in the first degree relatives was present in 10% of young patients. Resection was possible only in 50% young patients. 26% young patients underwent only palliative gastrojejunostomy. The most common operation was lower partial gastrectomy in 68%. Amongst the intraoperative findings peritoneal metastasis was seen in 17.4% in young patients. 50% young patients presented in stage IV as per AJCC classification (P value .004; sig.). None of the patients presented as stage 1 disease in young group. Conclusion. Early detection of stomach carcinoma is very important in all patients but in young patients it is of paramount importance. PMID:24381753

  3. Power to the patient.

    PubMed

    Hagland, M

    1996-10-20

    Getting and giving advice are two different things, especially when it comes to health care. Just ask Hamilton Jordan, former adviser to Jimmy Carter. When he was diagnosed with cancer, he didn't rely on anyone else's advice. What sets him apart from the average patient? The answer, increasingly, is not much. In fact Jordan personifies a new health care consumer: educated, interested, aware, and active in charting the course of his or her own health care. PMID:8897793

  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. Patient Safety in Surgery

    PubMed Central

    Makary, Martin A.; Sexton, J Bryan; Freischlag, Julie A.; Millman, E Anne; Pryor, David; Holzmueller, Christine; Pronovost, Peter J.

    2006-01-01

    Background: Improving patient safety is an increasing priority for surgeons and hospitals since sentinel events can be catastrophic for patients, caregivers, and institutions. Patient safety initiatives aimed at creating a safe operating room (OR) culture are increasingly being adopted, but a reliable means of measuring their impact on front-line providers does not exist. Methods: We developed a surgery-specific safety questionnaire (SAQ) and administered it to 2769 eligible caregivers at 60 hospitals. Survey questions included the appropriateness of handling medical errors, knowledge of reporting systems, and perceptions of safety in the operating room. MANOVA and ANOVA were performed to compare safety results by hospital and by an individual's position in the OR using a composite score. Multilevel confirmatory factor analysis was performed to validate the structure of the scale at the operating room level of analysis. Results: The overall response rate was 77.1% (2135 of 2769), with a range of 57% to 100%. Factor analysis of the survey items demonstrated high face validity and internal consistency (? = 0.76). The safety climate scale was robust and internally consistent overall and across positions. Scores varied widely by hospital [MANOVA omnibus F (59, 1910) = 3.85, P < 0.001], but not position [ANOVA F (4, 1910) = 1.64, P = 0.16], surgeon (mean = 73.91), technician (mean = 70.26), anesthesiologist (mean = 71.57), CRNA (mean = 71.03), and nurse (mean = 70.40). The percent of respondents reporting good safety climate in each hospital ranged from 16.3% to 100%. Conclusions: Safety climate in surgical departments can be validly measured and varies widely among hospitals, providing the opportunity to benchmark performance. Scores on the SAQ can serve to evaluate interventions to improve patient safety. PMID:16632997

  6. The patient with allergies.

    PubMed

    Franzese, Christine B; Burkhalter, Neal W

    2010-09-01

    Allergic disease affects a sizeable percentage of the general population, has a significant impact on patient quality of life, and exerts a significant financial burden on society. Atopic symptoms from inhalant allergens are among the most frequent complaints in outpatient medical visits. Key history and physical examination findings help to distinguish allergic rhinitis from other forms of chronic rhinosinusitis. Diagnostic testing may not be necessary unless immunotherapy is contemplated. PMID:20736101

  7. [The dementia patient caregiver].

    PubMed

    Bagnati, Pablo M

    2010-01-01

    Dementia results in an important economic, social and personal burden. To care for a patient with dementia can be a trascendent learning experience. At the same time, the caregiver's role can become strenuous physical and mental work. This article reviews the importance of assessing the caregiver from the moment of diagnostic work up, the stages the caregiver goes through in the disease evolution, and the "Caregiver syndrome" where the caregiver can become the "second victim" of dementia. PMID:21598739

  8. 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

  9. 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.

  10. [Echocardiography in elderly patients].

    PubMed

    Tabet, J-Y; Pascal, O; Monin, J-L

    2004-03-27

    IN THE CONTEXT OF AGEING: The Doppler echocardiography is a non-invasive technique that permits assessment of the "physiological" ageing of the cardiac and vascular structures, notably including a concentric remodelling of the left ventricle associated with relaxation abnormalities, dilatation of the left atrium, valvular reorganisation and a modification in the large vessels. IN A PATHOLOGICAL CONTEXT: The Doppler echocardiography also detects the various cardiovascular affections related to ageing: valvulopathies, notably calcified aortic stenosis and mitral failure due to mitral anulus calcification or prolapsus of the valve; primary hypertrophic cardiomyopathy or secondary to arterial hypertension or an amyloidosis, and possibly leading to heart failure with spared systolic function, frequent in elderly patients; ischemic cardiopathies that have benefited, as in younger patient, from new echographical stress testing techniques, which safely study the variability in myocardial ischemia. Transoesophageal echography can also be performed in elderly patients, but the indications of this more invasive and less well-tolerated examination must be assessed case by case. It is very useful when an intra-parietal aortic hematoma is suspected or during aortic dissection or infectious endocarditis. PMID:15105787

  11. [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

  12. 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

  13. [Tracheostomy in pediatrics patients].

    PubMed

    Fantoni, A; Ripamonti, D

    2002-05-01

    This is a case study of 79 children who under-went a tracheostomy with three different techniques, used in succession on three different groups of patients: surgical, percutaneous with progressive probes and translaryngeal technique. The pediatric patient, only under the age of 10, starts showing clear-cut anatomical differences compared to an adult, which gradually become more marked the younger the patient is. The causes of increased difficulties that can normally be found in these patients are obviously due to the reduced diameter of the airway and, above all, the high degree of pliability of the cartilaginous frame. After the discovery that even minimal external pressure can cause the collapse of the tracheal walls, it was decided to adopt the systematic use of a rigid bronchoscope, in order to be able to offer internal support to the anterior wall. The following advantages were obtained in the various techniques: In surgery it permitted the reduction, through the protrusion of the trachea, of the size of the operatory field, of the tracheal opening and consequently of the local trauma. It also caused less bleeding and reduced the risk of lesion to the pleural dome which are very frequent when a bronchoscope is not used. In the percutaneous method the bronchoscope allowed the application of the technique without complications, even in infants of only a few months' old, because it eliminated tracheal squashing, caused by the introduction of the needle, dilators and especially cannula, and the relative danger of lesion to the posterior wall of the trachea. This complication which was always impending in the original technique, which does not involve the use of a rigid bronchoscope, is the main reason which lead to the ban on PDT for patients younger than 16-18. In the translaryngeal method the advantages are of minor importance because they are limited to the initial stage of the procedure, the introduction of the needle and guide wire which are quicker and more precise. As regard the dilation, the TLT mode has a high level of intrinsic safety and is perfectly suitable to the anatomy of the child. In fact it is carried out from the inside towards the outside, causing a tendency to evertion of the tracheal wall which the physician must even limit with external pressure. It is remarkable that this contrast in pressure and the resulting compression of the peristomal tissues creates advantages, which are essential in younger children, that cannot be found in other tracheal techniques. These advantages are the complete absence of loss of blood, minimal local trauma and a perfect adherence of the stoma to the cannula, particularly effective at level of the tracheal wall. PMID:12029259

  14. Patients Satisfaction Evaluation

    PubMed Central

    Mutlu, Ebru Kaya; zdinler, Arzu Razak

    2014-01-01

    Objectives: Although many studies were focused on the assessment of patients' satisfaction, few studies have specifically addressed this issue for knee osteoartritis. Global rating of change (GRC) scales are very commonly used in clinical research, particularly in the musculoskeletal area. The purpose of this study was to evaluate the patients satisfaction of the Mulligans Mobilization with Movement (MWM) techniques, Passive Mobilization (PM) techniques and physical therapy modalities (PTM) (superficial heat or cold, Transcutaneous Electrical Nerve Stimulation (TENS) and therapeutic ultrasound) in subjects with osteoarthritis of the knee. Methods: Thirty subjects with bilateral osteoarthritis of the knee were randomly assigned to the MWM group (n=21) or the PM group (n=21) or PTM group (n=22). The duration of treatment was a maximum of 5 weeks (12 sessions) in all groups. The subjects satisfaction were assessed before and after treatment by the GRC scales. One-way analysis of variance was used to compare the groups. When significance was observed, pairwise post hoc tests were performed using Tukeys test. Results: Mean age and body mass index (BMI) of our study group were 547 years and 315 kg/m,respectively in the MWM group, 566 years and 314 kg/m,respectively in the PM group and 576 years and 325 kg/m,respectively in the PTM group There were no significant differences between the groups with respect to age, BMI and GRC before treatment (p>0.05). Patients satisfaction of the three groups after treatment were significantly different (F=6.732 p=0.002). Pairwise comparisons of the treatment groups revealed a significant difference in the patients satisfaction between MWM and PTM (p=0.007), and between PM and PTM (p=0.007); there was no significant difference between MWM and PM (p>0.05). Conclusion: Manual therapy seemed to be superior to physical therapy modalities for the patients satisfaction. Therefore, we suggest that physiotherapist either can apply MWM or PM techniques based on their clinical experience in the management of osteoarthritis of the knee.

  15. Mongolian spots: How important are they?

    PubMed Central

    Gupta, Divya; Thappa, Devinder Mohan

    2013-01-01

    Mongolian spots (MS) are congenital birthmarks seen most commonly over the lumbosacral area. They are bluish-green to black in color and oval to irregular in shape. They are most commonly found in individuals of African or Asian ethnic background. Although these lesions resolve by one to two years of age, widespread, extrasacral and dark colored MS sometimes persist into adulthood. Aberrant MS over occiput, temple, mandibular area, shoulders and limbs may be confused with other dermal melanocytoses and bruises secondary to child abuse, thus necessitating documentation at birth. Although traditionally believed to be benign in nature, they have now been shown to co-exist with inborn errors of metabolism, most commonly GM1 gangliosidosis and mucopolysaccharidosis type?I?(Hurlers disease), followed by mucopolysaccharidosis type II (Hunters syndrome), mucolipidosis, Niemann-Pick disease and mannosidosis. They have also been seen to co-exist with various vascular or other pigmented birthmarks like caf-au-lait macules. Co-existing Mongolian spots and vascular birthmarks like nevus flammeus, nevus anemicus or nevus spilus is termed as phakomatosis pigmentovascularis. This review focuses on the important associations of Mongolian spots and stresses upon the importance of screening babies with extensive MS. PMID:24340274

  16. Epstein-Barr virus-induced transformation of B cells for the diagnosis of genetic metabolic disorders--enumerative conditions for cryopreservation.

    PubMed

    Mello, A S; Burin, M G; Michellin, K; Viapiana, M; Giugliani, R; Coelho, J C; Bauer, M E

    2006-02-01

    Epstein-Barr virus (EBV) infection in vitro causes transformation of B cells and generates B lymphoblastoid cell lines (LCLs). These LCLs have been widely used for the diagnostic of several genetic metabolic disorders. However, up to now, efficiency of LCL generation has been based on misleading subjective analysis. In this study, quantitative analyses have been performed to indicate efficiency of B-cell transformation to measuring human lysosomal acid hydrolases associated with: GM1-gangliosidosis type I, Gaucher disease and mucopolysaccharidosis type I. Peripheral blood mononuclear cells were isolated from 13 subjects, and LCLs were produced by culturing them with EBV for 12 days. Activities of the enzymes beta-galactosidase, beta-glucosidase and alpha-iduronidase were measured before and after cryopreservation in liquid nitrogen for 30 days. Efficiency of the B-cell transformation was screened every 4 days by the enumeration of cell proliferation, cell counts and changes in granularity estimated by flow cytometry. We observed the generation of 13 LCLs. Cell transformation was confirmed by the gradual increase of cellular clusters, cell size and granularity. In addition, we determined that the activity of the enzymes mentioned above did not change following cryopreservation. These data suggest that our enumerative approach for screening of EBV-LCLs is efficient for the enzymatic determination of human lysosomal acid hydrolases and may thus replace misleading subjective analyses. PMID:16426420

  17. 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 ?-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, carbohydratedeficient 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

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

    PubMed Central

    Bonten, Erik J.; Annunziata, Ida; dAzzo, 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 ?-Galactosidase (?-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 ?-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

  19. Lysosomal multienzyme complex: pros and cons of working together.

    PubMed

    Bonten, Erik J; Annunziata, Ida; d'Azzo, Alessandra

    2014-06-01

    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 ?-galactosidase (?-GAL). Next to this 'core' complex, the existence of sub-complexes, which 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 ?-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. Patient Safety Threat - Syringe Reuse

    MedlinePLUS

    ... Injection Safety Share Compartir A Patient Safety Threat Syringe Reuse Important Information! Please read this fact sheet ... that you may be at risk due to syringe reuse by your healthcare provider. Patients need to ...

  1. Support for Patients and Families

    MedlinePLUS

    ... Patients and Families resources Support for Patients and Families Explore this section for support and advocacy organizations ... equipping advocates to become successful activists. Contact a Family Rare disorder team brings together groups, families, and ...

  2. Sleep Tips for Sjogren's Patients

    MedlinePLUS

    ... for Tips on Sleep for Sjgrens Patients. Dr. Fisher reminds patients that adequate sleep is especially important for those with Sjgrens syndrome, saying that sleep deprivation exacerbates daytime fatigue and ...

  3. 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

  4. 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

  5. Cutaneous abnormalities in uremic patients.

    PubMed

    Bencini, P L; Montagnino, G; Citterio, A; Graziani, G; Crosti, C; Ponticelli, C

    1985-01-01

    94 uremic patients, 68 on regular hemodialysis treatment (RHT) and 26 on chronic ambulatory peritoneal dialysis (CAPD), were followed for up to 20 months in search of dermatological manifestations. 79% of RHT and 76% of CAPD patients had some kind of cutaneous lesion. The most characteristic features of cutaneous involvement in RHT and CAPD patients were: cutaneous xerosis, pruritus, infectious manifestations and disorders of pigmentation. In 4 RHT patients, precancerous and cancerous manifestations were also observed. PMID:4010846

  6. 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

  7. 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

  8. What makes a patient unhappy.

    PubMed

    Tobin, Howard A

    2008-05-01

    The best way to avoid an unhappy postoperative patient is to do everything one can to optimize the preoperative consultation and select patients carefully. This article draws on the judgment of a cosmetic surgeon with years of experience. By focusing on the individual unique needs of each patient, and by not being afraid to say no, the wise surgeon can expect a very high satisfaction rate from patients. PMID:18355697

  9. Podcasting: contemporary patient education.

    PubMed

    Abreu, Daniel V; Tamura, Thomas K; Sipp, J Andrew; Keamy, Donald G; Eavey, Roland D

    2008-04-01

    Portable video technology is a widely available new tool with potential to be used by pediatric otolaryngology practices for patient and family education. Podcasts are media broadcasts that employ this new technology. They can be accessed via the Internet and viewed either on a personal computer or on a handheld device, such as an iPod or an MP3 player. We wished to examine the feasibility of establishing a podcast-hosting Web site. We digitally recorded pediatric otologic procedures in the operating room and saved the digital files to DVDs. We then edited the DVDs at home with video-editing software on a personal computer. Next, spoken narrative was recorded with audio-recording software and combined with the edited video clips. The final products were converted into the M4V file format, and the final versions were uploaded onto our hospital's Web site. We then downloaded the podcasts onto a high-quality portable media player so that we could evaluate their quality. All of the podcasts are now on the hospital Web site, where they can be downloaded by patients and families at no cost. The site includes instructions on how to download the appropriate free software for viewing the podcasts on a portable media player or on a computer. Using this technology for patient education expands the audience and permits portability of information. We conclude that a home computer can be used to inexpensively create informative surgery demonstrations that can be accessed via a Web site and transferred to portable viewing devices with excellent quality. PMID:18478793

  10. 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

  11. Turning patients over in bed

    MedlinePLUS

    ... arm across the chest. Cross the patient's upper ankle over the bottom ankle. If you are turning the patient onto the ... Make sure the patient's ankles, knees, and elbows are not resting on top of each other. Make sure the head and neck are in line ...

  12. [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

  13. 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

  14. 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

  15. 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

  16. 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

  17. Hyponatremia in cancer patients.

    PubMed

    Platania, Marco; Verzoni, Elena; Vitali, Milena

    2015-01-01

    Hyponatremia is the most frequent electrolyte disorder in hospitalized patients but also a well known poor prognostic factor in cancer patients. Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) is often misdiagnosed by oncologist because of difficulties in the interpretation of laboratory tests. Etiology is heterogeneous but the predominant cause is represented by the unbalance between excessive presence of water and serum sodium deficiency. Ectopic production of arginine vasopressin (AVP) develops more frequently in small cell lung cancer but it is not so rare in other malignancies. Neurological impairment may range from subclinical to life-threating symptoms depending by the rate of serum sodium deficiency. Appropriate diagnosis is essential to set a proper therapy. When hyponatremia is caused by SIADH, hypertonic saline infusion is indicated for acute presentation whereas fluid restriction is preferred in case of chronic asymptomatic evolution. Other options include vaptans, vasopressin receptor antagonists, targeted specifically for the correction of euvolemic hyponatremia. The aim of this brief report is to provide concise and specific informations for the management of SIADH in oncology clinical practice. PMID:25908038

  18. [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

  19. The Patient Who Falls

    PubMed Central

    Tinetti, Mary E.; Kumar, Chandrika

    2013-01-01

    Falls are common health events that cause discomfort and disability for older adults and stress for caregivers. Using the case of an older man who has experienced multiple falls and a hip fracture, this article, which focuses on community-living older adults, addresses the consequences and etiology of falls; summarizes the evidence on predisposing factors and effective interventions; and discusses how to translate this evidence into patient care. Previous falls; strength, gait, and balance impairments; and medications are the strongest risk factors for falling. Effective single interventions include exercise and physical therapy, cataract surgery, and medication reduction. Evidence suggests that the most effective strategy for reducing the rate of falling in community-living older adults may be intervening on multiple risk factors. Vitamin D has the strongest clinical trial evidence of benefit for preventing fractures among older men at risk. Issues involved in incorporating these evidence-based fall prevention interventions into outpatient practice are discussed, as are the trade-offs inherent in managing older patients at risk of falling. While challenges and barriers exist, fall prevention strategies can be incorporated into clinical practice. PMID:20085954

  20. 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

  1. [Neurological complications in cancer patients].

    PubMed

    Hundsberger, Thomas; Roth, Patrick; Roelcke, Ulrich

    2014-08-20

    Neurological symptoms in cancer patients have a great impact on quality of life and need an interdisciplinary approach. They lead to significant impairment in activities of daily living (gait disorders, dizziness), a loss of patients independency (vegetative disturbances, wheel-chair dependency) and interfere with social activities (ban of driving in case of epilepsy). In this article we describe three main and serious neurological problems in the context of oncological patients. These are chemotherapy-induced polyneuropathy, malignant spinal cord compression and epileptic seizures. Our aim is to increase the awareness of neurological complications in cancer patients to improve patients care. PMID:25146945

  2. 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

  3. [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

  4. Financial Distress in Cancer Patients

    PubMed Central

    de Souza, Jonas A.; Wong, Yu-Ning

    2013-01-01

    Novel diagnostic and therapeutic options offer hope to cancer patients with both localized and advanced disease. However, many of these treatments are often costly and even well-insured patients can face high out-of-pocket costs. Families may also be at risk of financial distress due to lost wages and other treatment-related expenses. Research is needed to measure and characterize financial distress in cancer patients and understand how it affects their quality of life. In addition, health care providers need to be trained to counsel patients and their families so they can make patient-centered treatment decisions that reflect their preferences and values. PMID:24349677

  5. Bibliotherapy in a Patients' Library *

    PubMed Central

    McDowell, David J.

    1971-01-01

    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

  6. Patient Perspectives on Biosimilar Insulin

    PubMed Central

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

    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

  7. Dysphagia in Tongue Cancer Patients

    PubMed Central

    Son, Yu Ri; Kim, Tae Gyun

    2015-01-01

    Objective To identify risk factors for dysphagia in tongue cancer patients. Dysphagia is a common complication of surgery, radiotherapy, and chemotherapy in tongue cancer patients. Previous studies have attempted to identify risk factors for dysphagia in patients with head and neck cancer, but no studies have focused specifically on tongue cancer patients. Methods This study was conducted on 133 patients who were diagnosed with tongue cancer and who underwent a videofluoroscopy swallowing study (VFSS) between January 2007 and June 2012 at the Asan Medical Center. Data collected from the VFSS were analyzed retrospectively. Patients with aspiration were identified. Results Patients showed a higher incidence of inadequate tongue control, inadequate chewing, delayed oral transit time, aspiration or penetration, vallecular pouch and pyriform residue, and inadequate laryngeal elevation after surgery. Moreover, male gender, extensive tumor resection, a higher node stage, and more extensive lymph node dissection were major risk factors for aspiration in tongue cancer patients. Conclusion Tongue cancer patients have difficulties in the pharyngeal phase as well as the oral phase of swallowing. These difficulties can worsen after tongue cancer surgery. Gender, the extent of tumor resection, and lymph node metastasis affect swallowing in tongue cancer patients. Physicians should take these risk factors into account when administering swallowing therapy to tongue cancer patients. PMID:25932417

  8. [Treatment of patients with osteoarthritis].

    PubMed

    Vargas Negrn, Francisco; Medina Abelln, Mara D; Hermosa Hernn, 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

  9. 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

  10. Thrombocytopenia in cancer patients.

    PubMed

    Liebman, Howard A

    2014-05-01

    Thrombocytopenia is a frequent complication of cancer and its treatment. The causes of thrombocytopenia in cancer patients can be diverse and multifactorial. Systemic chemotherapy is the most frequent cause of thrombocytopenia. The degree and duration thrombocytopenia depends upon whether the chemotherapeutic treatment is myeloablative, as used in stem cell transplants, or non-myeloablative, as typically used in solid non-hematologic malignancies. Additional causes of significant thrombocytopenia include tumor involvement of bone marrow and spleen; microangiopathic disorders such as disseminated intravascular coagulation, thrombotic thrombocytopenic purpura or hemolytic uremia syndrome. Lymphoproliferative malignancies can also be associated with secondary immune thrombocytopenia. Due to the broad differential diagnosis associated with cancer related thrombocytopenia, a careful diagnostic evaluation is indicated. The goal of treatment should be to maintain a safe platelet count to allow effective treatment of the underlying malignancy, prevent bleeding complications and to minimize the use of platelet product transfusion. PMID:24862148

  11. Patient literacy levels: a consideration when designing patient education programs.

    PubMed

    Wilson, F L; McLemore, R

    1997-01-01

    The purposes of this study were to examine (a) the relationship between patients' own reports of the highest grade completed in school and their actual reading level and (b) the relationship between literacy and the level of knowledge about self-care after patients had received education involving written discharge instructions. In addition, the content of the materials was analyzed for its cultural sensitivity. Twenty-six patients who had had either hip- or knee-replacement surgery at an inner-city hospital participated in this correlational descriptive study. There was a significant negative relationship between patients' own reports of highest grade completed in school and their actual reading level (r = -.39, p < .05). Rehabilitation nurses should find this study beneficial for developing, assessing, and using written patient education materials appropriate for the reading level of their patient populations. PMID:9416192

  12. 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

  13. [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

  14. [Tooth extraction on diabetic patients].

    PubMed

    Fragiskos, F; Massoulas, G B; Vagenas, N

    1990-02-01

    Performing extractions on diabetic patients is a complicated problem, which is connected with the type of diabetes and the various complications related to this disease. The main concern is to avoid acute incidents hyper or sub-glycemic comas during the operation and to secure a smooth post- operational course, namely an undisturbed post- extracture healing. In this study, 80 diabetic patients who came for extractions to the escodental department were divided into two categories: patients suffering from diabetes type I and patients suffering from diabetes type II. On the arrival of all patients, the contents of glycose in blood and acetone in urine were measured, and depending on the respective results patients of the first category were classified into three groups and patients of the second category were classified into four groups. Along with the normal measures usually taken for diabetic patients, specific instructions were given for the groups of each category, in order to prevent hyper- or sub-glycaminate incidents, during the operation. Proper instructions were further given for those patients, who had history chronical complications from diabetes. Extractions based on th method of classifying the patients into the above mentioned categories and groups have presented no complications whatever in our study. PMID:2151826

  15. 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

  16. 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

  17. Conversations with chronic schizophrenic patients.

    PubMed

    Morgan, R

    1979-02-01

    An account is given of some of the topics discussed during a small informal weekly open group meeting of chronic schizophrenic patients, based on occasional notes compiled over eleven years. The main feature of the patients' condition as displayed was poverty--clinical, social, behavioural, material and financial--and certain features suggested an organic aetiology. Reasons are given for considering that the patients' condition was predominantly caused by schizophrenia rather than by institutionalism. PMID:427336

  18. 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

  19. 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 ( ...

  20. 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

  1. 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

  2. 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

  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. [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

  5. 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

  6. 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

  7. Hearing thresholds in CAPD patients.

    PubMed

    Morton, L P; Reynolds, L; Zent, R; Rayner, B L

    1992-01-01

    Ultra high frequency (UHF, 10-20 kHz) and conventional audiometric thresholds (0,25-8 kHz) were obtained from 42 stable CAPD patients. Twenty-one (50%) and 11 (25%) of the patients had UHF and conventional hearing loss respectively, when compared to age related control data. This was unrelated to length of chronic renal failure and number of treatments with ototoxic drugs. Sixteen of these patients were monitored audiometrically using UHF during a course of vancomycin therapy for peritonitis. There was no significant change in hearing thresholds. In conclusion there is high incidence of hearing loss in CAPD patients which is unrelated to ototoxic drugs. PMID:1361774

  8. [Strongyloidiasis in nephrologic patients].

    PubMed

    Gravellone, Luciana; Battaglia, Carmelo; Caligara, Fabiana; D'Amato, Ippolita; Gandini, Eligio; Lucatello, Angelo; Rizzo, Maria Antonietta; Torpia, Regina; Brigante, Gioconda; Castiglioni, Alessandro

    2015-01-01

    Strongyloides stercoralis is a nematode causing strongyloidiasis, more frequent in immigrants and in travelers coming from tropical and subtropical areas. Infection is usually asymptomatic, frequently associated with eosinophilia. Immunocompromised patients are at high risk of developing hyperinfection syndrome (HI) or dissemination (SD), life threatening complications. Diagnosis of strongyloidiasis is firstly based on larvae isolation in stool samples; specific therapy involves the use of ivermectin as first choice and albendazole as second choice. We describe two cases of strongyloidiasis. The first one is a disseminated strongyloidiasis occurred in an Ecuadorian male on corticosteroid therapy for nephrotic syndrome due to focal segmental glomerulosclerosis, successfully treated with ivermectin; the second one involves another Ecuadorian male affected by acute kidney failure and nephrotic syndrome in IgA nephropathy with a diagnosis of chronic strongyloidiasis performed before starting the immunosuppressive treatment. The timing of treatment with ivermectin has allowed the complete eradication of the parasite before starting steroid and mycophenolate mofetil therapy, preventing the occurrence of a disseminated infection. Epidemiological data show us how strongyloidiasis is rising at our latitude because of increased number of migrants and travelers coming from endemic areas. So we must always exclude asymptomatic strongyloidiasis before prescribing a steroid or immunosuppressive therapy, in order to avoid developement of disseminated and often fatal disease. PMID:26005937

  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 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

  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. 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

  13. The Patient-Centered Medical Home and Patient Experience

    PubMed Central

    Martsolf, Grant R; Alexander, Jeffrey A; Shi, Yunfeng; Casalino, Lawrence P; Rittenhouse, Diane R; Scanlon, Dennis P; Shortell, Stephen M

    2012-01-01

    Objective To examine the relationship between practices' reported use of patient-centered medical home (PCMH) processes and patients' perceptions of their care experience. Data Source Primary survey data from 393 physician practices and 1,304 patients receiving care in those practices. Study Design This is an observational, cross-sectional study. Using standard ordinary least-squares and a sample selection model, we estimated the association between patients' care experience and the use of PCMH processes in the practices where they receive care. Data Collection We linked data from a nationally representative survey of individuals with chronic disease and two nationally representative surveys of physician practices. Principal Findings We found that practices' use of PCMH processes was not associated with patient experience after controlling for sample selection as well as practice and patient characteristics. Conclusions In our study, which was large, but somewhat limited in its measures of the PCMH and of patient experience, we found no association between PCMH processes and patient experience. The continued accumulation of evidence related to the possibilities of the PCMH, how PCMH is measured, and how the impact of PCMH is gauged provides important information for health care decision makers. PMID:22670806

  14. 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

  15. 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

  16. 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

  17. Patient Protection and Risk Selection

    PubMed Central

    Wynia, Matthew K; Zucker, Deborah; Supran, Stacey; Selker, Harry P

    2002-01-01

    BACKGROUND Individual physicians who are paid prospectively, as in capitated health plans, might tend to encourage patients to avoid or to join these plans according to the patient's health status. Though insurance risk selection has been well documented among organizations paid on a prospective basis, such physician-level risk selection has not been studied. OBJECTIVE To assess physician reports of risk selection in capitated health plans and explore potentially related factors. DESIGN AND PARTICIPANTS National mailed survey of primary care physicians in 19971998, oversampling physicians in areas with more capitated health plans. RESULTS The response rate was 63% (787 of 1,252 eligible recipients). Overall, 44% of physicians reported encouraging patients either to join or to avoid capitated health plans according to the patients' health status: 40% encouraged more complex and ill patients to avoid capitated plans and 23% encouraged healthier patients to join capitated plans. In multivariable models, physicians with negative perceptions of capitated plan quality, with more negative experiences in capitated plans, and those who knew at each patient encounter how they were being compensated had higher odds of encouraging sicker patients to avoid capitated plans (odds ratios, 2.0, 2.2, and 2.0; all confidence intervals >1). CONCLUSIONS Many primary care physicians report encouraging patients to join or avoid capitated plans according to the patient's health status. Although these physicians' recommendations might be associated primarily with concerns about quality, they can have the effect of insulating certain health plans from covering sicker and more expensive patients. PMID:11903774

  18. [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

  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. 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 <65years 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 (<65years) 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

  1. [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

  2. 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

  3. 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

  4. Idiom Comprehension in Aphasic Patients

    ERIC Educational Resources Information Center

    Papagno, Costanza; Tabossi, Patrizia; Colombo, Maria Rosa; Zampetti, Patrizia

    2004-01-01

    Idiom comprehension was assessed in 10 aphasic patients with semantic deficits by means of a string-to-picture matching task. Patients were also submitted to an oral explanation of the same idioms, and to a word comprehension task. The stimuli of this last task were the words following the verb in the idioms. Idiom comprehension was severely

  5. 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…

  6. 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

  7. Patient-centered Communication Research

    Cancer.gov

    Several recent reports, including NCI's Strategic Plan for Leading the Nation and the NCI-designated cancer center directors' report Accelerating Successes Against Cancer, have recognized the salience of patient-clinician communication in optimizing cancer patients' and survivors' health outcomes.

  8. Patient safety: the doctor's perspective.

    PubMed

    Shemesh, David; Olsha, Oded; Goldin, Ilya; Danin, Sigalit

    2015-01-01

    Medical errors can be defined as the failure of a planned action to be completed as intended or the use of a wrong plan to achieve an aim. Beyond their economic cost and their cost in human lives, errors cause loss of trust in the healthcare system by patients and diminished satisfaction by both patients and health professionals. There are many evidence-based safety-oriented behaviours and interventions that are easily implemented, such as ultrasound-guided central venous catheter insertion, prevention of catheter-related bloodstream infection and more. In vascular access, the development of research in patient safety has raised a variety of issues requiring study in order to provide the optimal patient safety approach. Patients are major contributors to their own safety, and as such, physicians should develop a new approach to involve them in the cycle of decision making through every step of their treatment. There are many opportunities along this path for the patient to be engaged in safety behaviours and for the access team to ensure such behaviours by employing simple strategies. The advent of the access centre, based on multidisciplinary teamwork, has enhanced the potential to improve patient safety by prevention of errors in planning and performing access surgery, avoiding delay in treatment of access malfunction and improving communication between the team members. However, a significant effort in research is still needed in order to implement intervention by evidence-based data focused on patient safety. PMID:25751565

  9. Patient Perspectives of Medical Confidentiality

    PubMed Central

    Sankar, Pamela; Mora, Susan; Merz, Jon F; Jones, Nora L

    2003-01-01

    OBJECTIVE To lay the groundwork for a better understanding of patient views on medical confidentiality. DESIGN Studies were found by searching medline, bioethicsline, and selected bibliographies. Articles concerning physician perspectives or implications of legal and administrative regulations were excluded. Only peer-reviewed journal articles reporting original research on patients' confidentiality views and conduct were included. MAIN RESULTS Many patients are unaware of or misunderstand their legal or ethical right to medical confidentiality protections, which leads them to both over- and underestimate confidentiality protections. The possibility that medical information might be revealed, intentionally or not, to acquaintances in a clinic or other social community troubles patients as much as information release to insurers or employers. A significant minority of patients distrust confidentiality protections, leading some to report that they delay or forgo medical care. If doubtful that confidentiality will be upheld, patients will act independently to protect information. CONCLUSIONS Our review found a wider variety of understandings and beliefs about medical confidentiality among patients than are often indicated in the writings of practitioners or legal experts. As medical confidentiality regulations evolve, these differences need to be recognized and accounted for in interactions between practitioners and patients. PMID:12911650

  10. Art Therapy with Laryngectomy Patients.

    ERIC Educational Resources Information Center

    Anand, Susan Ainlay; Anand, Vinod K.

    1997-01-01

    Reports on the experiences of patients with laryngeal cancer who used art therapy. Drawing on 14 years of experience and 109 laryngeal cancer patients, describes treatment results and the case material substantiating the distinct role of art therapy. Provides an overview of the special medical and therapeutic needs of this group. (RJM)

  11. 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

  12. 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.

  13. Preoperative Assessment of Geriatric Patients.

    PubMed

    Nakhaie, Mariam; Tsai, Andrea

    2015-09-01

    The preoperative assessment of geriatric patients provides an excellent opportunity to evaluate the patient for perioperative risk factors such as frailty, functional status, nutritional status, cardiovascular and pulmonary status, and substance dependence. It also provides an overall clinical picture on which health care providers can base a framework to reduce these risk factors. PMID:26315632

  14. 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.

  15. 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

  16. Rhinoplasty in Middle Eastern Patients.

    PubMed

    Sajjadian, Ali

    2016-01-01

    Rhinoplasty in patients of Middle Eastern origin requires complete understanding of nasal morphology and an individualized approach to create a racially congruent and aesthetically pleasing outcome. In this article, common anatomic features and characteristics and detailed steps, surgical techniques, and operative maneuvers that can lead to predictable outcome in rhinoplasty of Middle Eastern patients are discussed. PMID:26616714

  17. Industrial Robots for Patient Support

    NASA Astrophysics Data System (ADS)

    Sommer, Andres

    Flexibility, precision, and capability to support the clinical workflow make robots the ideal choice for ion beam therapy (IBT) facilities for use in patient positioning. To fulfill the clinical needs in IBT, an industrial robot patient positioner needs to be carefully designed regarding applications and safety issues as well as precision and handling.

  18. 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.

  19. 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…

  20. 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.

  1. 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

  2. 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

  3. [Patient adherence in antihypertensive treatment].

    PubMed

    Simonyi, Gbor

    2013-10-01

    Hypertension is one of the most frequent chronic diseases as well as most important cardiovascular risk factors in developed countries. Blood pressure control to target levels can significantly decrease the risk for development of coronary artery disease, stroke, chronic renal disease and mortality. Adequately maintained blood pressure is very important in high risk patients. In the treatment of hypertension, life style therapy and drug treatment have essential roles. Further, patient adherence plays a significant part of the treatment, too. Importantly, about half of the patients only become adherent to antihypertensive therapy by the end of the first year treatment. Hence, non-adherence of patients is an important cause for resistant hypertension. Adherence is influenced by the complexity of drug regimen and characteristic of drug class, as well as age and gender of patients. PMID:24077162

  4. Orthostatic Intolerance Ambulation in Patients Using Patient Controlled Analgesia

    PubMed Central

    Park, Kwang Ok

    2013-01-01

    Background Opioid analgesics are widely used to reduce postoperative pain and to enhance post-operative recovery. However, orthostatic intolerance (OI) induced by opioid containing intravenous patient controlled analgesia (IPCA) may hinder postoperative recovery. This study investigated factors that affect OI in patients receiving IPCA for postoperative pain control. Methods OI was instantly evaluated at the time of first ambulation in 175 patients taking opioid containing IPCA after open and laparoscopic subtotal gastrectomies. Patients were classified as having OI if they experienced dizziness, nausea/vomiting, blurred vision, headache, somnolence and syncope. Factors contributing to OI were assessed with logistic regression analysis. Results Out of 175 patients, 61 (52.6%) male and 44 (74.6%) female patients experienced OI at the time of first ambulation. The frequency of OI related symptoms were dizziness (97, 55.4%), nausea (46, 26.3%), headache (9, 5.1%), blurred vision (3, 1.7%) and vomiting (2, 1.1%). Significant risk factors for OI were gender (P=0.002) and total amount of opioids administered (P=0.033). Conclusions The incidence of OI is significantly higher in male than in female patients and is influenced by the opioid dose. PMID:23862002

  5. Iranian patients' perspective of patients' rights: a qualitative study.

    PubMed

    Khaledi, Shahnaz; Moridi, Golrokh; Valiee, Sina

    2016-01-01

    There is an increasing emphasis on "protecting patient rights", which has a great influence on the patient's well-being. This study aimed to explore patients' perspectives of patients' rights in the hospitals of Kurdistan University of Medical Sciences in Iran. This qualitative study used the content analysis method. The data were collected through in-depth interviews, conducted in Persian at the internal and surgical wards from 2012 to 2013. Consequently, interviews continued to be conducted on 20 patients, using content analysis, until data saturation. The findings highlighted aspects of patients' rights and five themes emerged from the interviews: having one's dignity respected, receiving care of the requisite quality, being shown financial consideration, receiving adequate information, and having a desirable and pleasant environment. The patients believed that for their rights to be upheld, it is necessary that together with the provision of enough facilities and equipment, they need to be respected and offered ideal healthcare services. This could be achieved by removing barriers and facilitating procedures. PMID:26826656

  6. Miglustat therapy in juvenile Sandhoff disease.

    PubMed

    Tallaksen, C M E; Berg, J E

    2009-12-01

    GM(2)-gangliosidosis is a rare and heterogeneous inherited metabolic disorder caused by autosomal recessive mutations in genes encoding the lysosomal enzyme ?-hexosaminidase, resulting in the accumulation of ganglioside GM(2) in various tissues, particularly the central nervous system. It is characterized by progressive neurological deterioration that mainly affects motor and spinocerebellar function. Several forms of GM(2)-gangliosidosis exist, including the Sandhoff variant. Currently there is no treatment for these conditions, except for palliative care. Miglustat (Zavesca) is a reversible inhibitor of glucosylceramide synthase, which catalyses the first committed step in the synthesis of glucose-based glycolipids. Miglustat has pharmacokinetic properties that allow it to cross the blood-brain barrier, and preclinical data suggest that it may benefit neuronopathic lysosomal storage diseases. Here we present a case report of a Norwegian patient with Sandhoff disease treated with miglustat at our centre in Norway. The patient initially presented with ataxia and dysarthria at 2-3 years of age, which progressed slowly during childhood. At age 14, he experienced episodes of depression and apathy, leading to weight loss. He was diagnosed with Sandhoff disease at age 16. Following 2.5 years of treatment with miglustat, his body weight was stabilized and disease progression appeared to have slowed, as evidenced by the lack of progressive brain atrophy. His depressive symptoms were managed using electroconvulsive treatment (ECT), which improved general functioning. These findings suggest that miglustat may provide beneficial effects in patients with juvenile Sandhoff disease, and that ECT may alleviate depressive symptoms. PMID:19898953

  7. 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

  8. The transition from 'informed patient' care to 'patient informed' care.

    PubMed

    Gardiner, Ruth

    2008-01-01

    We are in the midst of a real change in the application of information technology to support the delivery of healthcare. We are seeing a shift from the 'informed patient' which has resulted from improved access to healthcare information, primarily from the Web, to the 'participative patient' as we move into Web 2.0 territory. The last decade has seen significant strides in the application of healthcare information to support patient care including: Increased access to healthcare related information by the patient through access to healthcare information on the Web (1.0). The development of electronic patient/health records. Improved access to knowledge for care professionals has enabled the dissipation of professional clinical skills with the introduction of nurse practitioners and increased use of therapies. Improved access to patient related information across disciplines is beginning to enable the shift from acute based to community based care. The introduction of home care technologies has enabled self monitoring in supporting self care. There are also developments in the way care is provided with an increasing diversity of healthcare providers with the challenges this has presented in exchanging patient related information to support continuity of care. We are now at another major turning point that could present greater challenges for healthcare professionals, organisations and the patient or client. These developments include: The application of information sharing services commonly referred to as Web 2.0. As a result we are seeing a transition from the 'informed patient' to the 'participative patient' that will present increasing challenges for healthcare professionals and healthcare organisations in adapting care to embrace this evolution. New entrants to the ehealth market are now emerging such as Google and Microsoft who are competing to 'own' the 'healthcare consumer'. Open source solutions for EPR/EHRs are now emerging that will challenge the traditional mechanisms for delivery of organisational healthcare solutions. Technologies that have been growing in use and demand over the past decade are now being applied to healthcare including digital TV and mobile computing. What then are the challenges for patients, healthcare organisations and information service providers as we move from the passive role of the patient in the provision of their care to a more participative role? PMID:18560085

  9. 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

  10. Intralobar sequestration in adult patients.

    PubMed

    Berna, Pascal; Cazes, Aurlie; Bagan, Patrick; Riquet, Marc

    2011-06-01

    We examined retrospectively the characteristics and the outcomes of intralobar sequestrations (ILS). We reviewed data we obtained from the medical records of patients with ILS who underwent surgery at Laennec, Georges Pompidou European, and Amiens South Hospital. From 1985 to 2010, 26 consecutive adults patients underwent surgery for ILS. There were 14 males and 12 females. The average age was 37.3 years. The ILS was right-sided in 11 patients (42.3%) and left-sided in 15 patients (57.7%). A systemic artery supply was found during the preoperative period in 11. Surgery consisted of lobectomy (n=20), bilobectomy (n=1), segmentectomy (n=4), and pneumonectomy (n=1). There were no postoperative deaths, and the postoperative course was uneventful in 20 patients. All patients were alive and faring well at long-term follow-up (mean follow-up 36.5 7.2 months). Surgery consisted of lobectomy in most cases. The arterial supply came from the descending thoracic and abdominal aorta. Hemoptysis and/or recurrent infections were present in 14/26 (54%) of patients. These are the same symptoms as those leading to the diagnosis of bronchectasis. This suggests, for similar reasons, that ILS in adults should be nosologically very similar to acquired lesions, such as bronchectasis. PMID:21362733

  11. Social anxiety in orthognathic patients.

    PubMed

    Ryan, F S; Moles, D R; Shute, J T; Clarke, A; Cunningham, S J

    2016-01-01

    There is evidence that patients seeking orthognathic treatment may be motivated by social anxiety disorder (SAD). The aim of this study was to investigate SAD in orthognathic patients using the Brief Fear of Negative Evaluation Scale (BFNES) and to compare these findings with those of the general population. This was a cross-sectional, questionnaire study conducted in two parts. Firstly, a national survey was conducted to yield data for the BFNES from a large, random sample of the UK general population. Secondly, orthognathic patients completed the BFNES. The BFNES scores are reported in two formats: the original 12-item scale (O-BFNES) and a shorter eight-item version (S-BFNES). With regards to the national survey, 1196 individuals participated. The mean O-BFNES score was 29.72 (standard deviation (SD) 9.39) and S-BFNES score was 15.59 (SD 7.67). With regards to the orthognathic sample, 61 patients participated. The mean O-BFNES score was 39.56 (SD 10.35) and the mean S-BFNES score was 24.21 (SD 8.41). Orthognathic patients had significantly higher scores than the general UK population (P<0.001), and multiple linear regression revealed that age, gender, and patient status were all independent predictors of BFNES scores. From the results of this study, orthognathic patients experience significantly higher levels of social anxiety than the general population. PMID:26304605

  12. 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

  13. 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

  14. 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

  15. 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

  16. 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

  17. Optimal nutrition in hemodialysis patients.

    PubMed

    Ikizler, T Alp

    2013-03-01

    Protein-energy wasting (PEW) is highly prevalent in patients undergoing maintenance hemodialysis (MHD). It is important to note that 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 of 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 efficiently use the gastrointestinal tract. 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

  18. Data Management for Cardiac Patients

    PubMed Central

    Iltis, R.; Benzing, G.; Jaspers, G.

    1981-01-01

    A system for management of medical data, storage and retrieval using a desk top calculator is described. The system is economical, flexible, and can be expanded to store data for an unlimited number of patients. Presently the design is planned for storage of data on 10,000 patients covering a ten year period. Management of data is governed by two types of flexible disks and a magnetic tape: (a) Directory disks holding about 1,250 patients' names, hospital number, dates, etc. (Eight disks are sufficient for 10,000 patients), (b) Patients' data disks containing 315 charts per disk with names, personal data, diagnostic and surgical codes and dates. Thirty-two disks are required for 10,000 patients, (c) Magnetic tape built-in into the calculator used for initialization, updating diagnostic codes, and as a back up for the disk. Recently our storage capacity has been increased by the use of a dual disk 9895A with 2M byte storage capability. This change permits the number of directory disks be reduced to 2 and the number of patients' disks to 8. ImagesFigure 2

  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. 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

  1. 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

  2. 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…

  3. 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

  4. Synergistic approach to patient dialysate

    PubMed Central

    Dragotoiu, A; Checheri??, AI; Cioclteu, A; Rizeanu, S

    2015-01-01

    The stress a patient is subjected to during dialysis treatment can be reduced by using a synergetic approach by the medical team. The integration into therapy of the positive psychical resources such as: active positive coping mechanisms, individual or family mental resilience, improvement of the image and self-esteem, better tolerance to frustration can represent an important part in the improvement of the patients quality of life, determination of a positive approach of the situations both for him and close friends and relatives. PMID:26361514

  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. 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

  7. Respiratory Emergencies in Geriatric Patients.

    PubMed

    Tyler, Katren; Stevenson, Dane

    2016-02-01

    Acute dyspnea in older patients is a common presentation to the emergency department. Acute dyspnea in older adults is often the consequence of multiple overlapping disorders, such as pneumonia precipitating acute heart failure. Emergency physicians must be comfortable managing patients with acute dyspnea of uncertain cause and varying goals of care. In addition to the important role noninvasive ventilation (NIV) plays in full resuscitation, NIV can be useful as a method of providing supportive or nearly fully supportive care while more information is gathered from the patients and their loved ones. PMID:26614240

  8. Patient risk from interproximal radiography

    SciTech Connect

    Gibbs, S.J.; Pujol, A. Jr.; Chen, T.S.; Malcolm, A.W.; James, A.E. Jr.

    1984-09-01

    Computer simulation methods for determining patient dose from dental radiography have demonstrated that patient risk from a two-film interproximal examination ranges from 1.1 X 10(-8) to 3.4 X 10(-7) using 90-kVp beams, depending on film speed, projection technique, and age and sex of the patient. Further, changing from a short-cone round-beam to a long-cone technique with rectangular collimation reduces risk by a factor of 2.9, independent of other factors.

  9. 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

  10. 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

  11. 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

  12. 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 antibodymediated complement activation in the pathogenesis and clinical course of MMN. PMID:26161430

  13. Dealing with the difficult patient.

    PubMed Central

    Smith, S.

    1995-01-01

    Dealing with difficult patients can represent a significant burden in the life of doctors. It is more productive, however, to view this burden as a product of the interaction between doctor and patient, for which both have a responsibility, rather than attributing any problems encountered to shortcomings of the patient alone. There is a significant risk in such situations of potentially harmful over-medicalisation. It behoves doctors, therefore, to try to prevent such problems becoming established, or make some attempt to rectify matters if they have already become so. Much is known about the factors that contribute to successful and unsuccessful clinical transactions. The awareness of what doctors bring both as professionals and as individual people to this interaction, will count as much as the practical clinical efforts made towards helping patients. PMID:7494768

  14. Implant rehabilitation in bruxism patient.

    PubMed

    Goiato, Marcelo Coelho; Sonego, Mariana Vilela; dos Santos, Daniela Micheline; da Silva, Emily Vivianne Freitas

    2014-01-01

    A white female patient presented to the university clinic to obtain implant retained prostheses. She had an edentulous maxillary jaw and presented three teeth with poor prognosis (33, 34 and 43). The alveolar bone and the surrounding tissues were healthy. The patient did not report any relevant medical history contraindicating routine dental treatment or implant surgery, but self-reported a dental history of asymptomatic nocturnal bruxism. The treatment plan was set and two Branemark protocols supported by six implants in each arch were installed after a 6-month healing period. A soft occlusal splint was made due to the patient's history of bruxism, and the lack of its use by the patient resulted in an acrylic fracture. The prosthesis was repaired and the importance of using the occlusal splint was restated. In the 4-year follow-up no fractures were reported. PMID:24907215

  15. 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

  16. Hospitalized Patients and Fungal Infections

    MedlinePLUS

    ... Diseases Mycotic Diseases Branch Hospitalized Patients and Fungal Infections Recommend on Facebook Tweet Share Compartir Even though ... hospitalized. What you need to know about fungal infections Fungal infections can range from mild to life- ...

  17. Bathing a patient in bed

    MedlinePLUS

    Bed bath; Sponge bath ... Some patients cannot safely leave their beds to bathe. For these people, daily bed baths can help keep their skin healthy, control odor, and increase comfort. If moving the ...

  18. Should Immunocompromised Patients Have Pets?

    PubMed Central

    Steele, Russell W.

    2008-01-01

    Purpose: To evaluate the risks and benefits of pet ownership by immunodeficient patients, focusing primarily on organisms that colonize animals and are transmitted to humans. Those diseases that are known to be progressive or more severe in patients with altered immune function are emphasized. Methods: A review of the medical and veterinary literature pertaining to zoonoses transmitted by domestic animals was completed. Information pertaining to issues involving immunosuppressed patients including AIDS was carefully evaluated and summarized for inclusion. Results: There are significant clinical and psychosocial benefits to pet ownership. However, numerous diseases can be acquired from these animals which may be more severe in immunocompromised individuals. Conclusion: Simple guidelines for pet ownership by immunosuppressed patients can be implemented to reduce their risk of disease and allow them to safely interchange with their pets. PMID:21603465

  19. 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

  20. 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.

  1. 38 CFR 17.33 - Patients' rights.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Patients' rights. 17.33... Patient Rights 17.33 Patients' rights. (a) General. (1) Patients have a right to be treated with dignity... privacy with regard to their personal needs. (2) Patients have a right to receive, to the extent...

  2. 38 CFR 17.33 - Patients' rights.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Patients' rights. 17.33... Patient Rights 17.33 Patients' rights. (a) General. (1) Patients have a right to be treated with dignity... privacy with regard to their personal needs. (2) Patients have a right to receive, to the extent...

  3. 38 CFR 17.33 - Patients' rights.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Patients' rights. 17.33... Patient Rights 17.33 Patients' rights. (a) General. (1) Patients have a right to be treated with dignity... privacy with regard to their personal needs. (2) Patients have a right to receive, to the extent...

  4. 38 CFR 17.33 - Patients' rights.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Patients' rights. 17.33... Patient Rights 17.33 Patients' rights. (a) General. (1) Patients have a right to be treated with dignity... privacy with regard to their personal needs. (2) Patients have a right to receive, to the extent...

  5. 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.

  6. 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

  7. 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

  8. Hypnotherapy and the Suicidal Patient

    PubMed Central

    Glick, Daniel

    1973-01-01

    Three cases are presented in which a family physician treats a suicidal patient by the hypnotherapeutic method described. It is postulated that such a method may be quick, effective, and relatively safe method for a diagnostic and therapeutic approach in family practice. In the hypnotic state, the patient is hypersuggestible and can accept suggestions which meet her unconscious needs to be loved as an individual within the milieu of the ego state at the time her psychopathology occurred. PMID:20468906

  9. 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

  10. [Several patients with memory disorders].

    PubMed

    Walstra, G J; Teunisse, S

    1997-02-22

    In three patients, referred to a memory clinic because of memory impairment, three different types of memory dysfunction were observed: a deficit in encoding of new episodic memories, a relatively pure loss of semantic memory and an impairment in retrieval of stored information. These patients were diagnosed as suffering from Alzheimer's disease, semantic dementia and frontotemporal dementia respectively. The diagnosis was essential in determining the individual management strategy. PMID:9157294

  11. Is My Patient An Athlete?

    PubMed Central

    Bullard, Jack

    1974-01-01

    This article outlines the ways in which the practice of medicine is affected by patients' participation in sports, or indeed any form of physical exercise. Several instances are cited where the patient's need for a quick recovery will materially affect the treatment he is given. The family physician must be aware of these instances, from the post coronary exercise program to premenstrual tension in an Olympic athlete. PMID:20469053

  12. Sedation in critically ill patients.

    PubMed

    Oldham, Mark; Pisani, Margaret A

    2015-07-01

    Sedation in the intensive care unit (ICU) is a topic that has been frequently researched, and debate still exists as to what are the best sedative agents for critically ill patients. There is increasing interest in sleep and circadian rhythm disturbances in the ICU and how they may impact on outcomes. In addition to patient-related and ICU environmental factors that likely impact sleep and circadian rhythm in the ICU, sedative and analgesic medications may also play a role. PMID:26118921

  13. Patient management of pulmonary embolism

    SciTech Connect

    Gilworth, D.L.; Donovan, B.C.; Morrison, R.; Ryan, K.; Reagan, K.; Goldhaber, S.Z.

    1988-03-01

    This is the first article in a four-part continuing education series addressing patient care and the clinical management of disease. This series is not directed at nuclear medicine procedures themselves, but focuses on topics related to patients referred for nuclear medicine studies. After reading this article, the reader should be able to: 1) discuss the diagnosis of pulmonary embolism; and 2) discuss conventional versus thrombolytic approaches to therapy.

  14. Patient selection: indications and contraindications.

    PubMed

    Choy, D S; Botsford, J; Black, W A

    1995-06-01

    Patient selection is critical to the success of PLDD. In general, the herniation must have continuity with the parent disc; rupture of the annulus is not a contraindication. Several other orthopedic conditions constitute absolute or relative contraindications. All patients must be individualized. Our criteria for inclusion is undergoing continuing change. What is unacceptable now may, with modifications, become acceptable in the future. It is important not to adopt a fixed position at this early stage of PLDD. PMID:10150640

  15. [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

  16. Balance billing: the patients' perspective.

    PubMed

    Kifmann, Mathias; Scheuer, Florian

    2011-01-01

    We study the effects of 'balance billing', i.e., allowing physicians to charge a fee from patients in addition to the fee paid by Medicare. First, we show that on pure efficiency grounds the optimal Medicare fee under balance billing is zero. An active Medicare policy thus can only be justified when distributional concerns are accounted for. Extending the analysis by Glazer and McGuire, we therefore analyze the optimal policy from the patients' point of view. We demonstrate that, from the patients' perspective, a positive fee can be superior under balance billing. Furthermore, patient welfare can be lower if balance billing is prohibited. In particular, this is the case if the administrative costs of Medicare are large. However, we cannot rule out that prohibiting balance billing may be superior. Finally, we show that payer fee discrimination increases patient welfare if Medicare's administrative costs are high or if Medicare's optimal fee under balance billing implies lower quality for fee-only patients.JEL-classification: I11, I18, H51. PMID:22827899

  17. Balance billing: the patients' perspective

    PubMed Central

    2011-01-01

    We study the effects of 'balance billing', i.e., allowing physicians to charge a fee from patients in addition to the fee paid by Medicare. First, we show that on pure efficiency grounds the optimal Medicare fee under balance billing is zero. An active Medicare policy thus can only be justified when distributional concerns are accounted for. Extending the analysis by Glazer and McGuire, we therefore analyze the optimal policy from the patients' point of view. We demonstrate that, from the patients' perspective, a positive fee can be superior under balance billing. Furthermore, patient welfare can be lower if balance billing is prohibited. In particular, this is the case if the administrative costs of Medicare are large. However, we cannot rule out that prohibiting balance billing may be superior. Finally, we show that payer fee discrimination increases patient welfare if Medicare's administrative costs are high or if Medicare's optimal fee under balance billing implies lower quality for fee-only patients. JEL-classification: I11, I18, H51 PMID:22827899

  18. Hepatitis C in hemodialysis patients

    PubMed Central

    Marinaki, Smaragdi; Boletis, John N; Sakellariou, Stratigoula; Delladetsima, Ioanna K

    2015-01-01

    Despite reduction of hepatitis C prevalence after recognition of the virus and testing of blood products, hemodialysis (HD) patients still comprise a high risk group. The natural history of hepatitis C virus (HCV) infection in dialysis is not fully understood while the clinical outcome differs from that of the general population. HD patients show a milder liver disease with lower aminotransferase and viral levels depicted by milder histological features on liver biopsy. Furthermore, the silent clinical course is consistent with a slower disease progression and a lower frequency of cirrhosis and hepatocellular carcinoma. Potential explanations for the beneficial impact of uremia and hemodialysis on chronic HCV infection are impaired immunosurveillance leading to a less aggressive host response to the virus and intradialytic release of hepatoprotective cytokines such as interferon (IFN)-? and hepatocyte growth factor. However, chronic hepatitis C is associated with a higher liver disease related cardiovascular and all-cause mortality of HD patients. Therapy is indicated in selected patients groups including younger patients with low comorbidity burden and especially renal transplant candidates, preferably after performance of a liver biopsy. According to current recommendations, choice of treatment is IFN or pegylated interferon with a reported sustained viral response at 30%-40% and a withdrawal rate ranging from 17% to 30%. New data regarding combination therapy with low doses of ribavirin which provide higher standard variable rates and good safety results, offer another therapeutic option. The new protease inhibitors may be the future for HCV infected HD patients, though data are still lacking. PMID:25848478

  19. 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

  20. 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

  1. 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

  2. ETHICAL MODELS OF PHYSICIAN--PATIENT RELATIONSHIP REVISITED WITH REGARD TO PATIENT AUTONOMY, VALUES AND PATIENT EDUCATION.

    PubMed

    Borza, Liana Rada; Gavrilovici, Cristina; Stockman, Ren

    2015-01-01

    The present paper revisits the ethical models of patient--physician relationship from the perspective of patient autonomy and values. It seems that the four traditional models of physician--patient relationship proposed by Emanuel & Emanuel in 1992 closely link patient values and patient autonomy. On the other hand, their reinterpretation provided by Agarwal & Murinson twenty years later emphasizes the independent expression of values and autonomy in individual patients. Additionally, patient education has been assumed to join patient values and patient autonomy. Moreover, several authors have noted that, over the past few decades, patient autonomy has gradually replaced the paternalistic approach based on the premise that the physician knows what is best for the patient. Neither the paternalistic model of physician-patient relationship, nor the informative model is considered to be satisfactory, as the paternalistic model excludes patient values from decision making, while the informative model excludes physician values from decision making. However, the deliberative model of patient-physician interaction represents an adequate alternative to the two unsatisfactory approaches by promoting shared decision making between the physician and the patient. It has also been suggested that the deliberative model would be ideal for exercising patient autonomy in chronic care and that the ethical role of patient education would be to make the deliberative model applicable to chronic care. In this regard, studies have indicated that the use of decision support interventions might increase the deliberative capacity of chronic patients. PMID:26204658

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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.

  8. [Nutritional support in stroke patients].

    PubMed

    Burgos Pelez, Rosa; Segurola Gurrutxaga, Hegoi; Bretn Lesmes, Irene

    2014-01-01

    Stroke is a public health problem of the first order. In developed countries is one of the leading causes of death, along with cardiovascular disease and cancer. In addition, stroke is the leading cause of permanent disability in adulthood. Many of the patients who survive do so with significant sequelae that limit them in their activities of daily living. Most strokes (80-85%) are due to ischemia, while the rest are hemorrhagic. We have identified many modifiable risk factors, some with an important relationship with dietary factors or comorbidities in wich the diet has a significant impact. The incidence of malnutrition in stroke patients is not well known, but most likely impacts on patient prognosis. Furthermore, the nutritional status of patients admitted for stroke often deteriorates during hospitalization. It is necessary to perform a nutritional assessment of the patient in the early hours of admission, to determine both the nutritional status and the presence of dysphagia. Dysphagia, through alteration of the safety and efficacy of swallowing, is a complication that has an implication for nutritional support, and must be treated to prevent aspiration pneumonia, which is the leading cause of mortality in the stroke patient. Nutritional support should begin in the early hours. In patients with no or mild dysphagia that can be controlled by modifying the texture of the diet, they will start oral diet and oral nutritional supplementation will be used if the patient does not meet their nutritional requirements. There is no evidence to support the use of nutritional supplements routinely. Patients with severe dysphagia, or decreased level of consciousness will require enteral nutrition. Current evidence indicates that early nutrition should be initiated through a nasogastric tube, with any advantages of early feeding gastrostomy. Gastrostomy will be planned when the enteral nutrition support will be expected for long-term (4 weeks). Much evidence points to the importance of glycemic control during hospitalization for stroke. Hyperglycemia at diagnosis and during the first hours of admission impact on patient prognosis. The goal of glycemic control necessary to modify this bad prognosis without adding risk by iatrogenic hypoglycemia is still matter of debate. PMID:25077346

  9. 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 ...

  10. Depression and Anxiety in Migraine Patients

    MedlinePLUS

    ... Depression and Anxiety in Migraine Patients Print Email Depression and Anxiety in Migraine Patients ACHE Newsletter Sign ... newsletter by entering your e-mail address below. Depression and Anxiety in Migraine Patients Todd A. Smitherman, ...

  11. Giving a Voice to Patients and Caregivers.

    PubMed

    Thomason, Melissa; Toman, Margaret; Potter, Matthew

    2015-01-01

    A key part of patient and family engagement involves listening to patients and keeping their perspective in mind. This article provides a chance for patients and family caregivers to tell their stories. PMID:26510220

  12. Insight among psychotic patients with auditory hallucinations.

    PubMed

    Lera, Guillem; Herrero, Neus; Gonzlez, Jose; Aguilar, Eduardo; Sanjun, Julio; Leal, Carmen

    2011-07-01

    Poor insight in psychosis has been described as a seeming lack of awareness of the deficits, consequences of the disorder, and of the need for treatment. The aim of this study is to investigate whether patients with auditory hallucinations have less insight than those without hallucinations, and to determine which hallucination characteristics are related to patient insight. Using the PANSS and PSYRATS, the authors have evaluated the lack of insight data corresponding to 168 psychotic patients divided into three groups: patients with a history of nonpersistent hallucinations, patients with persistent hallucinations, and patients without hallucinations. Patients with persistent hallucinations showed significantly less insight than patients without persistent hallucinations and patients without hallucinations, the farther away the hallucination is located, the greater the lack of patient insight. Patients who hear the hallucination inside their head rather than outside show better insight, possibly because such patients can understand the voice as being created by their own mind. PMID:21448990

  13. 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

  14. 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

  15. 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.

  16. Treating drug-dependent patients in hospitals.

    PubMed

    Skene, Loane; Keays, David; Gardner, Bruce

    2002-08-01

    Are hospital staff legally permitted to test drug-dependent patients for drugs or infectious disease without the patient's consent in order to treat the patient or to protect themselves or other patients? What should staff do with "suspicious" items in the patient's possession (drugs, credit cards in different names, firearms)? Can drug-dependent patients lawfully use illicit drugs in hospital? Who should supply and administer them? PMID:12242876

  17. 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

  18. 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

  19. How to assess deteriorating patients.

    PubMed

    Barker, Mike; Rushton, Melanie; Smith, Joyce

    2015-11-11

    Rationale and key points This article aims to help practitioners to undertake the assessment of deteriorating patients using a standardised ABCDE (airway, breathing, circulation, disability, exposure) approach that allows the practitioner to assess the patient in a structured and timely manner, and to identify the specific needs of the patient using the look, listen and feel method. ? Systematic assessment is central to recognising a deteriorating patient and to taking timely and appropriate action to minimise the incidence of further deterioration. ? The ABCDE approach can be used to identify patients at risk of becoming acutely unwell and to summon appropriate assistance at an early stage. ? The practitioner should review obtained physiological data to develop an effective management plan and indicate future investigations. Reflective activity Clinical skills articles can help update your practice and ensure it remains evidence based. Apply this article to your practice. Reflect on and write a short account of: 1. What you have gained from reading this article. 2. How this knowledge will influence your practice. Subscribers can upload their reflective accounts at: rcni.com/portfolio . PMID:26554995

  20. 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

  1. 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).

  2. Bedside Evaluation of Dizzy Patients

    PubMed Central

    Huh, Young-Eun

    2013-01-01

    In recent decades there has been marked progress in the imaging and laboratory evaluation of dizzy patients. However, detailed history taking and comprehensive bedside neurotological evaluation remain crucial for a diagnosis of dizziness. Bedside neurotological evaluation should include examinations for ocular alignment, spontaneous and gaze-evoked nystagmus, the vestibulo-ocular reflex, saccades, smooth pursuit, and balance. In patients with acute spontaneous vertigo, negative head impulse test, direction-changing nystagmus, and skew deviation mostly indicate central vestibular disorders. In contrast, patients with unilateral peripheral deafferentation invariably have a positive head impulse test and mixed horizontal-torsional nystagmus beating away from the lesion side. Since suppression by visual fixation is the rule in peripheral nystagmus and is frequent even in central nystagmus, removal of visual fixation using Frenzel glasses is required for the proper evaluation of central as well as peripheral nystagmus. Head-shaking, cranial vibration, hyperventilation, pressure to the external auditory canal, and loud sounds may disclose underlying vestibular dysfunction by inducing nystagmus or modulating the spontaneous nystagmus. In patients with positional vertigo, the diagnosis can be made by determining patterns of the nystagmus induced during various positional maneuvers that include straight head hanging, the Dix-Hallpike maneuver, supine head roll, and head turning and bending while sitting. Abnormal smooth pursuit and saccades, and severe imbalance also indicate central pathologies. Physicians should be familiar with bedside neurotological examinations and be aware of the clinical implications of the findings when evaluating dizzy patients. PMID:24285961

  3. [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

  4. Mismanagement of a hypochondriacal patient

    PubMed Central

    Bidaki, Reza; Mahmoudi, Maryam; Khalili, Behrang; Abedi, Mostafa; Golabbakhsh, Aryan; Haghshenas, Alireza; Sadeghi, Ali; Tabibian, Seyed Reza; Mirhosseini, Seyyed Mohammad Mahdy

    2015-01-01

    Hypochondriasis is a persistent preoccupation that despite appropriate medical evaluations and assurance of patient's physical health, the patient insists on having a serious disease. The case which is discussed in this article is a 39-years-old woman that hospitalized for half of her life and no one can perceive her disorder according to her assertions. The mentioned case is a difficult patient with fear of oxygen shortage and being choked (Pnigophobia) which leads to continuous tendency to wear oxygen device even during sleep. There is no benefit in exaggerating her condition for herself so there is no fictitious disorder considered. During the therapy she has been assured that she does not have a serious disease and she has learnt to decrease oxygen intake and breath with his mouth. The point that makes this study different from the others is that most of hypochondriacal patients have a fear of getting HIV, cancers, hepatitis and MS but our patient has phobia of pulmonary embolism. PMID:25709989

  5. 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 555 % 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

  6. Bedside evaluation of dizzy patients.

    PubMed

    Huh, Young-Eun; Kim, Ji-Soo

    2013-10-01

    In recent decades there has been marked progress in the imaging and laboratory evaluation of dizzy patients. However, detailed history taking and comprehensive bedside neurotological evaluation remain crucial for a diagnosis of dizziness. Bedside neurotological evaluation should include examinations for ocular alignment, spontaneous and gaze-evoked nystagmus, the vestibulo-ocular reflex, saccades, smooth pursuit, and balance. In patients with acute spontaneous vertigo, negative head impulse test, direction-changing nystagmus, and skew deviation mostly indicate central vestibular disorders. In contrast, patients with unilateral peripheral deafferentation invariably have a positive head impulse test and mixed horizontal-torsional nystagmus beating away from the lesion side. Since suppression by visual fixation is the rule in peripheral nystagmus and is frequent even in central nystagmus, removal of visual fixation using Frenzel glasses is required for the proper evaluation of central as well as peripheral nystagmus. Head-shaking, cranial vibration, hyperventilation, pressure to the external auditory canal, and loud sounds may disclose underlying vestibular dysfunction by inducing nystagmus or modulating the spontaneous nystagmus. In patients with positional vertigo, the diagnosis can be made by determining patterns of the nystagmus induced during various positional maneuvers that include straight head hanging, the Dix-Hallpike maneuver, supine head roll, and head turning and bending while sitting. Abnormal smooth pursuit and saccades, and severe imbalance also indicate central pathologies. Physicians should be familiar with bedside neurotological examinations and be aware of the clinical implications of the findings when evaluating dizzy patients. PMID:24285961

  7. Neurovascular disturbances in headache patients.

    PubMed

    Drummond, P D; Lance, J W

    1984-01-01

    The prevalence of icecream headache, icepick-like pains and premonitory symptoms (changes in mood, appetite and alertness one to 24 hours before headache onset) was studied in 530 patients referred for neurological assessment of headache. Pain in the head after icecream or cold drinks was reported by 37% of patients, more often by those whose headaches were associated with focal neurological symptoms and gastrointestinal disturbances. The affected region coincided with that of the customary headache in 33% of patients questioned. Icepick-like pains were reported by 39% of patients and were restricted to the distribution of the customary headache in 40% of these. Premonitory symptoms were noted by 30% of patients, particularly those with focal neurological symptoms before or during the attack. Premonitory symptoms may form part of a diffuse cerebral or hypothalamic disturbance preceding headache, while the prevalence of icecream headache and icepick-like pains suggests that pain pathways and neurovascular reflexes to sensory stimulation are hyperexcitable even between headache episodes. These phenomena favour a neurological origin of migrainous headache. PMID:6568949

  8. Mismanagement of a hypochondriacal patient.

    PubMed

    Bidaki, Reza; Mahmoudi, Maryam; Khalili, Behrang; Abedi, Mostafa; Golabbakhsh, Aryan; Haghshenas, Alireza; Sadeghi, Ali; Tabibian, Seyed Reza; Mirhosseini, Seyyed Mohammad Mahdy

    2015-01-01

    Hypochondriasis is a persistent preoccupation that despite appropriate medical evaluations and assurance of patient's physical health, the patient insists on having a serious disease. The case which is discussed in this article is a 39-years-old woman that hospitalized for half of her life and no one can perceive her disorder according to her assertions. The mentioned case is a "difficult patient" with fear of oxygen shortage and being choked (Pnigophobia) which leads to continuous tendency to wear oxygen device even during sleep. There is no benefit in exaggerating her condition for herself so there is no fictitious disorder considered. During the therapy she has been assured that she does not have a serious disease and she has learnt to decrease oxygen intake and breath with his mouth. The point that makes this study different from the others is that most of hypochondriacal patients have a fear of getting HIV, cancers, hepatitis and MS but our patient has phobia of pulmonary embolism. PMID:25709989

  9. 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

  10. Clinical pharmacogenetics in pediatric patients.

    PubMed

    Husain, Anwar; Loehle, Jennifer A; Hein, David W

    2007-10-01

    Pediatric pharmacogenetic studies have the potential to improve the quality of medical care for children. The pediatric population presents a unique pharmacogenetic challenge as children have the additional complexity of ontological phenotypes that impact their drug response. Prescribing medications in children has historically been largely empirical, but utilization of pharmacogenetic information will allow pediatricians to gain key information regarding which patients are best suited for a particular therapeutic agent and which patients may be at risk for serious potentially life-threatening complications from standard treatment regimens. Although large, prospective, multisite investigators are still needed, we illustrate selective clinical examples of the pharmacogenetics for treatment of transplantation, asthma, leukemia and attention-deficit hyperactivity disorder in pediatric patients. PMID:17979513

  11. 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

  12. 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

  13. Understanding hypoglycemia in hospitalized patients

    PubMed Central

    Hulkower, Raphael D; Pollack, Rena M; Zonszein, Joel

    2014-01-01

    SUMMARY Controlling blood glucose in hospitalized patients is important as both hyperglycemia and hypoglycemia are associated with increased cost, length of stay, morbidity and mortality. A limiting factor in stringent control is the concern of iatrogenic hypoglycemia. The association of hypoglycemia with mortality has led to clinical guideline changes recommending more conservative glycemic control than had previously been suggested, with the use of patient specific approaches when appropriate. Healthier, stable patients may be managed with stricter control while the elderly and severely ill may be managed less aggressively. While the avoidance of hypoglycemia is essential in clinical practice, recent studies suggest that a higher mortality rate occurs in spontaneous rather than iatrogenic hypoglycemia. Therefore, inpatient hypoglycemia may be viewed more as a biomarker of disease rather than a true cause of fatality. PMID:25197322

  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. [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

  16. 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

  17. 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

  18. 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

  19. 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

  20. Major trauma in geriatric patients.

    PubMed Central

    Champion, H R; Copes, W S; Buyer, D; Flanagan, M E; Bain, L; Sacco, W J

    1989-01-01

    Contemporary trauma to the elderly, its severity and associated mortality and morbidity in 111 United States and Canadian trauma centers are described. Three-thousand eight-hundred thirty-three (3,833) trauma patients age 65 years or older are compared to 42,944 injured patients under age 65. Although both groups had equivalent measures of injury severity, the older group had higher case fatality and complication rates and longer hospital stays. The results raise important questions regarding the triage, acute care, accurate prediction of outcome, and hospital reimbursement for the elderly injured patient, with implications for care evaluation, quality assurance, and the long-term viability of trauma centers and systems of care. PMID:2764207

  1. 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

  2. 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

  3. 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

  4. [Medication review for dementia patients].

    PubMed

    Jahns, Karl-Peter; Kilimann, Ingo; Ritter, Christoph A; Hoffmann, Wolfgang; Teipel, Stefan J; Fiss, Thomas

    2012-03-01

    Due to demographic changes we are faced with several challenges as an increasing prevalence of dementia patients. We report on a medication review of a patient with Alzheimer's disease as well as Lewy body dementia. The intake of risperidone was interrupted instead of a dose reduction which was recommended by the psychiatrist to improve mobility. As an adverse event the patient developed serious psychiatric symptoms which were treated in an acute care facility. We discussed several alternative treatment options (pipamperon, melperon, haloperidol, risperidone, clozapine, olanzapine, aripiprazol, and quetiapin) in a case conference. Due to a short half life period and insignificant anticholinergic effects we decided to choose quetiapin. Despite a small number of taken drugs we identified several potential drug related problems which were solved in a multipartite health care professional team. PMID:22452126

  5. Hyponatremia in critically ill patients

    PubMed Central

    Padhi, Rajesh; Panda, Baikuntha Nath; Jagati, Snehalata; Patra, Subhas Chandra

    2014-01-01

    Context: Hyponatremia is a common electrolyte disturbance in critically ill hence understanding its implications is important. Aims: This study was carried out to ascertain frequency, predisposing conditions and outcome in critically ill patients with hyponatremia on intensive care unit (ICU) admission. Settings and Design: This was an observational, prospective study of a series of ICU patients during a 12-month period. Materials and Methods: The patients were divided into two groups: Hyponatremic (serum sodium < 135 mmol/L) and Eunatremic groups (135-145 mmol/L). Clinical examination included volume status and drug history, biochemistries, clinical diagnosis and cause of hyponatremia. Statistical Analysis Used: Fisher's exact test, unpaired t-tests Wilcoxon ranksum tests, profile-likelihood method, log-rank test and KaplanMeier curves were used. P < 0.05 were considered to be statistically significant. Results: In the hyponatremic group, the frequency of hyponatremia on ICU admission was 34.3%, most were euvolumic, 58.96%. Females comprised of 36.5%. The mean age was 60.4 17.2. The Syndrome of inappropriate Antidiuretic Hormone (SIADH) criteria was met in ninety-one patients (36.25%), peumonia being the leading cause of SIADH. Patients with severe sepsis, elective surgery patients, renal failure and heart failure, cirrhosis of liver and subarachnoid hemorrhage were other more likely etiologic causes (P < 0.05). The hyponatremic group spent a longer time in the ICU (P = 0.02), had longer mechanical ventilator days (P < 0.05) and had an increased mortality rate (P = 0.01). Conclusions: Hyponatremia present on admission to the ICU is independent risk factors for poor prognosis. PMID:24678150

  6. [Malignancies in patients on dialysis].

    PubMed

    Motn, J; Krzek, M; Fnek, J; Mukensnabl, P

    2005-01-01

    Incidence of malignancies in patients on dialysis is higher than in the comparable population. The topic is discussed from different points of view: A. Malignancy as a cause of renal failure (renal and urinary tract tumors, von Hippel-Lindau disease, Wilms tumor, multiple myeloma, tumors that compress urinary tract). B. Treatment of malignancies may result in renal failure and dialysis (nephrectomy, tumor-lysis syndrome, postradiation fibrosis, direct toxic effect of chemotherapy). C. Dialyzed patients are in higher risk of malignancies, especially those of the kidney and urinary tract but also of pharynx and larynx, thyroid gland etc. The following factors may play some roles: the basic disease, (e.g. analgesic and Balcan nephropathies, China Herba nephropathy etc.), changed metabolic milieu with retention of carcinogens, deficiency of selenium and other substances, acquired renal cysts, compromised immunity, decreased "wash-effect" in oligo-anuria and possible influence of dialysis itself (contact with phtalates, ethylenoxide, nitrosamines etc.). D. Special problems in diagnostics of malignancies. Controversial validity of s.c. "tumor markers" is mentioned. Among the causes of death in dialyzed patients cardiovascular and infectious diseases predominate. The active search for renal and urinary tract tumors should be performed. All other diagnostic procedures depend on the individual patient's risk profile. E. Methods of renal substitution are used in the treatment of malignancies (e.g. dialysis in the tumor-lysis syndrome, plasma filtration to remove paraproteins, intraperitoneal administration of chemotherapy similar to peritoneal dialysis approach). F. Malignant tumors and dialysis--some ethical problems. Withdrawal of dialysis in severely suffering patients should be approved by an informed patient and followed by maximal palliative therapy including palliative ultrafiltration if threat of lung edema occurs. PMID:16013514

  7. 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

  8. 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

  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. Redefining Retention: Recovery from the Patients Perspective

    PubMed Central

    Mitchell, Shannon Gwin; Morioka, Rika; Reisinger, Heather Schacht; Peterson, James A.; Kelly, Sharon M.; Agar, Michael H.; Brown, Barry S.; OGrady, Kevin E.; Schwartz, Robert P.

    2011-01-01

    This study examines the process of discharge and treatment reentry for six participants who entered treatment voluntarily but were administratively discharged from methadone treatment programs. The participants completed semistructured interviews at treatment entry and at four, eight and 12 months post-treatment entry. Grounded theory methodology was used to examine the phenomenon of treatment reentry from the perspective of the patients, who often viewed their recovery as an accumulation of positive changes. Differences in terms of the patients goals and motivations for seeking treatment from those of the treatment programs, combined with difficulties encountered during the treatment process eventually led to discharge. However, these patients were then able to navigate their way through the treatment system in different ways in order to remain in treatment. The authors conclude that failure to abide by treatment clinic rules do not necessary constitute treatment failure from the perspective of patients, who often wish to remain in treatment even if it is not progressing optimally from the programs perspective. As a result, the recovery process can be more fragmented and is often characterized by a series of cyclical treatment episodes rather than continuous time in treatment, thereby impeding their progress towards recovery. PMID:21858956

  11. Psychiatric Patients' Perspective: Nursing Uniforms.

    PubMed

    Kk, Leyla; mez, Tuba; Kaar, Selma; Smeli, Fatma; Ta?k?ran, zgl

    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

  12. 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

  13. [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

  14. 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.

  15. [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

  16. [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

  17. Improving Patient's Primary Medication Adherence

    PubMed Central

    Leguelinel-Blache, Graldine; Dubois, Florent; Bouvet, Sophie; Roux-Marson, Clarisse; Arnaud, Fabrice; Castelli, Christel; Ray, Valrie; 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?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

  18. 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

  19. [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 Universittsklinikum 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

  20. Patients' perceptions of patient education on psychiatric inpatient wards: a qualitative study.

    PubMed

    Htnen, H; Suhonen, R; Warro, H; Pitknen, A; Vlimki, M

    2010-05-01

    This study describes patients' perceptions of different types of patient education interventions and areas where patient education should be improved on psychiatric wards. Thematic interviews were conducted with 16 patients who had completed the information technology (IT)-based patient education, conventional patient education with leaflets or patient education according to ward standards during their hospital stay. Data were analysed using inductive content analysis. Patients' perceptions of patient education varied depending on which patient education group they had participated in. Patients participating in IT-based or conventional patient education perceived education as a systematic and planned process. However, especially patients in the patient education group applying ward standard education perceived patient education as occasional information dissemination situations. To improve patient education, patients suggested that it should be based on their individual needs and offered with different methods systematically to all patients. The results indicate that patients find structured and systematic patient education programmes useful. Different educational methods should be used, not forgetting interaction between patient and nurse, which was reportedly as an essential element of patient education. PMID:20529184

  1. The Use of Patients as Student Evaluators.

    ERIC Educational Resources Information Center

    Henkin, Yaakov; And Others

    1991-01-01

    A comparison of patient (n=181) and tutor (n=31) evaluations of medical student (n=41) behavior in an internal medicine clerkship revealed difficulties with patient evaluation of students. The process is time consuming and introduces tension between students and patients, and patients are poor discriminators. (Author/MSE)

  2. 29 CFR 525.4 - Patient workers.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... WITH DISABILITIES UNDER SPECIAL CERTIFICATES 525.4 Patient workers. With respect to patient workers... 29 Labor 3 2013-07-01 2013-07-01 false Patient workers. 525.4 Section 525.4 Labor Regulations... disabilities normally perform, in whole or in part in the institution or elsewhere. However, a patient does...

  3. 29 CFR 525.4 - Patient workers.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... WITH DISABILITIES UNDER SPECIAL CERTIFICATES 525.4 Patient workers. With respect to patient workers... 29 Labor 3 2010-07-01 2010-07-01 false Patient workers. 525.4 Section 525.4 Labor Regulations... disabilities normally perform, in whole or in part in the institution or elsewhere. However, a patient does...

  4. 29 CFR 525.4 - Patient workers.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... WITH DISABILITIES UNDER SPECIAL CERTIFICATES 525.4 Patient workers. With respect to patient workers... 29 Labor 3 2011-07-01 2011-07-01 false Patient workers. 525.4 Section 525.4 Labor Regulations... disabilities normally perform, in whole or in part in the institution or elsewhere. However, a patient does...

  5. 29 CFR 525.4 - Patient workers.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... WITH DISABILITIES UNDER SPECIAL CERTIFICATES 525.4 Patient workers. With respect to patient workers... 29 Labor 3 2014-07-01 2014-07-01 false Patient workers. 525.4 Section 525.4 Labor Regulations... disabilities normally perform, in whole or in part in the institution or elsewhere. However, a patient does...

  6. 21 CFR 880.2720 - Patient scale.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Patient scale. 880.2720 Section 880.2720 Food and... Patient scale. (a) Identification. A patient scale is a device intended for medical purposes that is used to measure the weight of a patient who cannot stand on a scale. This generic device includes...

  7. 21 CFR 880.2720 - Patient scale.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Patient scale. 880.2720 Section 880.2720 Food and... Patient scale. (a) Identification. A patient scale is a device intended for medical purposes that is used to measure the weight of a patient who cannot stand on a scale. This generic device includes...

  8. 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.

  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. Transforming consumer health informatics through a patient work framework: connecting patients to context

    PubMed Central

    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

  11. 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 recordbased 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

  12. Cancer Patients and Fungal Infections

    MedlinePLUS

    ... in cancer patients: an international autopsy survey. European Journal of Clinical Microbiology & Infectious Diseases 1992;11:99-109. Pagano L, Akova M, Dimopoulos G, Herbrecht R, Drgona L, Blijlevens N. Risk assessment and ... Journal of Antimicrobial Chemotherapy 2011;66:i5-i14. Ribaud ...

  13. 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

  14. Haematuria in Postrenal Transplant Patients

    PubMed Central

    Wang, Ziting; Vathsala, Anantharaman; Tiong, Ho Yee

    2015-01-01

    Haematuria has a prevalence of 12% in the postrenal transplant patient population. It heralds potentially dangerous causes which could threaten graft loss. It is important to consider causes in light of the unique, urological, and immunological standpoints of these patients. We review the literature on common causes of haematuria in postrenal transplant patients and suggest the salient approach to the evaluation of this condition. A major cause of haematuria is urinary tract infections. There should be a higher index of suspicion for mycobacterial, fungal, and viral infection in this group of immunosuppressed patients. Measures recommended in the prevention of urinary tract infections include early removal of foreign bodies as well as prophylactic antibiotics during the early transplant phase. Another common cause of haematuria is that of malignancies, in particular, renal cell carcinomas. When surgically managing cancer in the setting of a renal transplant, one has to be mindful of the limited retropubic space and the need to protect the anastomoses. Other causes include graft rejections, recurrences of primary disease, and calculus formation. It is important to perform a comprehensive evaluation with the aid of an experienced multidisciplinary transplant team. PMID:25918706

  15. Patient Compliance and Medication Perception.

    ERIC Educational Resources Information Center

    Buckalew, L. W.; Sallis, R. E.

    1986-01-01

    Addresses problem of patient noncompliance with regimens of prescribed medication, with attention to incidence and illogical nature of this behavior. Psychological theory of cognitive dissonance is suggested as appropriate to understanding some aspects of noncompliance because medicinal preparations represent stimuli that are not necessarily

  16. Patient privacy and social media.

    PubMed

    Hader, Amy L; Brown, Evan D

    2010-08-01

    Healthcare providers using social media must remain mindful of professional boundaries and patients' privacy rights. Facebook and other online postings must comply with the Health Insurance Portability and Accountability Act of 1996 (HIPAA), applicable facility policy, state law, and AANA's Code of Ethics. PMID:20879626

  17. Exercise for the Overweight Patient.

    ERIC Educational Resources Information Center

    Work, Janis A.

    1990-01-01

    Exercise can help patients maintain lean body mass during weight loss. Although exercise is not extremely useful in shedding excess pounds, it helps keep off weight lost through calorie restriction. This article discusses the specifics of exercise prescription, types of exercise, motivation to exercise, and special problems such as diabetes. (SM)

  18. Hypertension in Patients with Cancer

    PubMed Central

    de Souza, Vinicius Barbosa; Silva, Eduardo Nani; Ribeiro, Mario Luiz; Martins, Wolney de Andrade

    2015-01-01

    There is a known association between chemotherapy and radiotherapy for treatment of cancer patients and development or worsening of hypertension. The aim of this article is to review this association. A literature search was conducted for articles reporting this association on the databases PubMed, SciELO and LILACS between 1993 and 2013. There was a high coprevalence of hypertension and cancer, since both diseases share the same risk factors, such as sedentary lifestyle, obesity, smoking, unhealthy diet and alcohol abuse. The use of chemotherapy and adjuvant drugs effective in the treatment of cancer increased the survival rate of these patients and, consequently, increased the incidence of hypertension. We described the association between the use of angiogenesis inhibitors (bevacizumab, sorafenib and sunitinib), corticosteroids, erythropoietin and non-steroidal anti-inflammatory drugs with the development of hypertension. We also described the relationship between hypertension and carotid baroreceptor injury secondary to cervical radiotherapy. Morbidity and mortality increased in patients with cancer and hypertension without proper antihypertensive treatment. We concluded that there is need for early diagnosis, effective monitoring and treatment strategies for hypertension in cancer patients in order to reduce cardiovascular morbidity and mortality. PMID:25742420

  19. Sex Guilt in Abortion Patients

    ERIC Educational Resources Information Center

    Gerrard, Meg

    1977-01-01

    A measure of sex guilt was administered to clients of a university problem pregnancy counseling service who were planning to have abortions and to a group of sexually active nonpregnant university coeds. Sex guilt was found to be significantly higher for the abortion patients than for the nonpregnant group. (Author)

  20. 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

  1. [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

  2. [Maintaining patients' autonomy at home].

    PubMed

    Niang, Bndicte; 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

  3. BAYESIAN BICLUSTERING FOR PATIENT STRATIFICATION.

    PubMed

    Khakabimamaghani, Sahand; Ester, Martin

    2016-01-01

    The move from Empirical Medicine towards Personalized Medicine has attracted attention to Stratified Medicine (SM). Some methods are provided in the literature for patient stratification, which is the central task of SM, however, there are still significant open issues. First, it is still unclear if integrating different datatypes will help in detecting disease subtypes more accurately, and, if not, which datatype(s) are most useful for this task. Second, it is not clear how we can compare different methods of patient stratification. Third, as most of the proposed stratification methods are deterministic, there is a need for investigating the potential benefits of applying probabilistic methods. To address these issues, we introduce a novel integrative Bayesian biclustering method, called B2PS, for patient stratification and propose methods for evaluating the results. Our experimental results demonstrate the superiority of B2PS over a popular state-of-the-art method and the benefits of Bayesian approaches. Our results agree with the intuition that transcriptomic data forms a better basis for patient stratification than genomic data. PMID:26776199

  4. [Psychological support to burn patients].

    PubMed

    Vlastelica, Mirela

    2014-10-01

    Severe burns and their treatment are among the most painful experiences a person can have. Emotional needs of burn patients have long been overshadowed by the focus on survival. Today, when the survival rate is much higher than in the past, the need of psychological and psychosocial engagement in working with victims of severe burns has emerged. A patient undergoing various stages of adjustment is faced with emotional challenges that accompany physical recovery. Adapting to burn injury involves a complex interplay between patient characteristics before the occurrence of burn, environmental factors, and the nature of the burns and medical care required. Adaptation implies adoption of new ideas about themselves and their body, new body image and new self image. Psychiatric and psychological treatment must be incorporated in burn treatment centers within a multidisciplinary treatment team. Psychology and psychotherapy should address the problem of loss, grief, acceptance of body image and self image, in terms of psychiatric conditions of delirium, acute stress disorder, posttraumatic stress disorder, anxiety, depression and other psychiatric disorders. Technical assistance and support should be provided to the patient family members. In some cases, psychosocial treatment never ends; it takes years, later related to rehabilitated burns. PMID:25326989

  5. Patient Safety in Clinical Trials

    Cancer.gov

    Information for patients, their families and friends, and the general public about how the rights and safety of people who take part in clinical trials are protected. Learn about informed consent, institutional review boards (IRB's), and how trials are closely monitored for safety.

  6. 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

  7. [Care of the terminal patient].

    PubMed

    Buisn, R; Delgado, J C

    2007-01-01

    In the work of health professions there have always been those who believed that the important thing was to treat all the patients until the final consequences, but there are also those who have considered that the important thing was to treat the patient and when this was not possible, to care for him until the end. Amongst the latter was Cicely Saunders, founder of the Hospices movement. She believed that in the terminal phase of the disease, when there was an increase in the deterioration the patient was to suffer, which also had a great impact on the family and team treating him/her, the aim should change and be replaced by care as the only goal. That care should be redirected towards providing comfort to the patient and his/her family. Because in this phase each positive action carried out on one of them is taken as something that is also positive for the other. The health team must transform itself into a care team. Care as a means and the quality of life and comfort as an end are must be our aims as carers during the final phase of life, as announced by the European Sub-committee of Palliative Care of the EEC, May 5th 1991, when defining palliative care. PMID:18227884

  8. 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

  9. [DGRW-update: patient education].

    PubMed

    Faller, H; Reusch, A; Meng, K

    2011-10-01

    Patient education programmes, i.?e. standardized, manualized, interactive group programmes aiming to increase self-management and empowerment, are a core element of medical rehabilitation for chronic conditions. In an update of the evidence of the effectiveness of patient education, its effectiveness was proven for a broad spectrum of chronic disorders, such as diabetes mellitus, chronic low back pain, rheumatoid arthritis, coronary heart disease, chronic heart failure, bronchial asthma, COPD, and cancer, as well as for the modification of health behaviours, such as diet and exercise. To sustain effects, aftercare interventions, such as support provided by phone, were found to be successful. Interventions targeted to particular patient groups according to gender, age, or migration background are also being developed more frequently. When evaluating educational interventions not only distal outcomes, such as quality of life and participation, should be used but also proximal outcomes such as self-management skills. A recent survey of patient education practice in medical rehabilitation revealed a continuing potential for optimization relative to manualization, evaluation and didactics. However, the dissemination of innovative programmes into rehabilitation routine presents a major challenge. PMID:21976261

  10. Uninvited "guests" in patient homes.

    PubMed

    Hall, Mellisa A

    2008-03-01

    This article reviews commonly prescribed medications used to treat insect bites or stings in a case study format. The review describes the implications of treatment for a patient with chronic kidney disease and acute hepatic obstruction who experienced an insect bite. PMID:18332740

  11. Brucellosis in Egyptian female patients.

    PubMed

    Sabah, Ahmed A; Aly, Ahmad M; Tawab, Ahmad H Abdel; Arafa, Wafaa A S

    2008-08-01

    Over six months, 129 consecutive brucellosis cases were diagnosed in females attending the outpatients' clinics the females in Al-Azhar and Ain Shams Universities Hospitals. Their ages ranged between 12-65 years old. 113 (87.6%) gave history of raw milk consumption, 13 (10%) gave history of home slaughtering of sheep, 2 (1.5%) gave history of animal contact, and one patient gave history of abortion, that partner had brucellosis. A total of 61.2% of patients gave serum agglutination test of 1: 640, who suffered acute or subacute infection. Titers of 1:320 (38.8%) were found in the majority of chronic cases. Causes of endemic parasitosis were excluded. Symptoms were fever (79.5%), headache (72.4%), generalized arthralgia (65.3%), sweating (65.3%), chills (63.8%), backache (34.6%), abdominal pain (27.5%), loss of appetite (25.5%), lassitude (17.2%), myalgia (14.2%), monoarthralgia (7.9%). Spinal involvement was in 15% patients, who had chronic brucellosis. 32/35 were successfully treated with a combination of streptomycin and tetracycline, 17/21 with streptomycin and septrin, 38/43 with tetracycline and septrin, and 26/26 (100%) with rifampicin and tetracycline or septrin, which treated all resistant patients. PMID:18853637

  12. Screening pneumonia patients for mimivirus.

    PubMed

    Dare, Ryan K; Chittaganpitch, Malinee; Erdman, Dean D

    2008-03-01

    Acanthamoeba polyphaga mimivirus (APM), a virus of free-living amebae, has reportedly caused human respiratory disease. Using 2 newly developed real-time PCR assays, we screened 496 respiratory specimens from 9 pneumonia-patient populations for APM. This virus was not detected in any specimen, which suggests it is not a common respiratory pathogen. PMID:18325263

  13. Patients, friends, and relationship boundaries.

    PubMed Central

    Rourke, J. T.; Smith, L. F.; Brown, J. B.

    1993-01-01

    When patient and physician are close friends, both professional and personal relationships can suffer. Jointly exploring and setting explicit boundaries can help avoid conflict and maintain these valuable relationships. This is particularly important when the physician practises in a small community where such concurrent relationships are unavoidable. PMID:8292931

  14. Nutrition support of HIV+ patients.

    PubMed

    Dwyer, J T

    1991-01-01

    Case management strategies for the nutritional support of patients infected with the human immunodeficiency virus (HIV) are evolving as the disease becomes less rapidly fatal and more chronic. Nutritional status changes in advanced HIV infection are similar in many respects to protein-calorie malnutrition. Current clinical effort and research focuses on the beneficial effects of preserving lean body mass and keeping asymptomatic patients in good nutritional status by preventing micronutrient deficiencies and by treating preexisting nutritional problems rather than attempting to intervene late in the disease's course, after secondary malnutrition has already developed. Nutrition support and intervention trials only late in the disease process have not been promising in reversing weight loss once it has occurred. Special diets, such as lactose- or gluten-free diets, may be helpful in some cases as asymptomatic treatment of some opportunistic infections, and such measures may slow additional losses. However, secretory diarrhea, which often seems to be inherent to the disease itself, is not ameliorated by such measures. Current research is focusing on the potential role of glutamine in slowing malabsorption and on combinations of diet and drug treatments. Asymptomatic patients are now the focus of concern. Preserving good nutritional status by attention to preventing weight loss and loss of lean body mass and assuring food safety are primary. Symptomatic patients require specific assistance depending on the presence of opportunistic infections and the drugs required. Specific nutrition support measures depend on whether or not the gut is functional.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1856104

  15. Patient satisfaction in prosthodontic treatment: multidimensional paradigm.

    PubMed

    Sangappa, Sunila Bukanakere

    2012-03-01

    A number of different factors contribute to an efficient clinical outcome in prosthetic dentistry. Differences between patient's and prosthodontist's perception of treatment display great variability. Patient satisfaction in prosthetic dentistry is a multidimensional concept as is patient's perception of dental care. Patient satisfaction can be assessed if it is carefully defined. In the prosthodontic treatment context patient satisfaction can be expected to interact with the patient's entire life situation. This article highlights the issues that reflect the different dimensions of patient satisfaction in prosthodontic care. PMID:23448832

  16. Patient safety: the what, how, and when.

    PubMed

    Albrecht, Roxie M

    2015-12-01

    Patient safety is a construct that implies behavior intended to minimize the risk of harm to patients through effectiveness and individual performance designed to avoid injuries to patients from the care that is intended to help them. The Accreditation Council for Graduate Medical Education has made patient safety a focused area in the new Clinical Learning Environment Review process. This lecture will focus on definitions of patient safety terminology; describe the culture of patient safety and a just culture; discuss what to report, who to report it too, and methods of conducting patient safety investigations. PMID:26522775

  17. [Patients' rights--doctors' duties].

    PubMed

    Jaeger, L; Bertram, E; Grate, S; Mischkowsky, T; Paul, D; Probst, J; Scala, E; Wbllenweber, H D

    2015-06-01

    On 26 February 2013 the new "Law on Patients' Rights" (hereinafter also the "Law") became effective. This Law strengthens patients' rights vis--vis the insurdnce company and also regulates patients' rights regarding their relation to the doctor. This has consequences for the laws on medical liability all doctors must consider. The doctor's performance is and remains a service and such service does not hold any guarantee of success. Nevertheless, this Law primarily reads as a "law on the duties of physicians". To duly take into account these duties and to avoid mistakes and misinterpretation of the Law, the Ethics Committee of the Consortium of Osteosynthesis Trauma Germany (AOTRAUMA-D) has drafted comments on the Law. Brief summaries of its effects are to be found at the end of the respective comment under the heading "Consequences for Practice". The text of the law was influenced particularly by case law, as continuously developed by the German Federal Court of Justice ("BGH"). The implementation of the Law on Patients' Rights was effected by the newly inserted sections 630a to 630h of the German Civil Code (the "BGB"), which are analysed below. The following comments are addressed to physicians only and do not deal with the specific requirements and particularities of the other medical professions such as physiotherapy, midwifery and others so on. Special attention should be paid to the comments on the newly inserted Duty to inform, which has to be fullfilled prior to any diagnostic or therapeutic procedure (sec. 630c para 2 sentence 1 BGB). Under certain conditions the doctor also has to inform the patient about the circumstances that lead to the presumed occurance of a therapeutic or diagnostic malpractice (sec. 630c para. 2 sentence 2 BGB), based on the manifestation of an undesired event or an undesired outcome. As before, the patient's valid consent to any procedure (sec. 630d BGB) is directly linked to the comprehensive and timely provision of information (sec. 630e BGB). Comprehensive documentation obligations regarding all procedures are stipulated in sec. 630f BGB. As before, the burden of proof still rests with the patient, unless a severe malpractice has been established (sec. 630h BGB). The definition of "severe malpractice" remains unchanged and is based on the case law of the Federal Court of Justice (BGH). The patient's obligations to preserve his or her health and to actively support the process of recovery and securing a positive outcome of the treatment are not explicitly mentioned in the Law. Nevertheless, the patient and the physician need to work closely together to achieve a successful result of the treatment. In case the patient does not give his or her cooperation, the physician should consider terminating the treatment relationship. PMID:26281286

  18. Patient-reported outcomes in clinical practice.

    PubMed

    Dobrozsi, Sarah; Panepinto, Julie

    2015-12-01

    Patient-reported outcome (PRO) measurement plays an increasingly important role in health care and understanding health outcomes. PROs are any report of a patient's health status that comes directly from the patient, and can measure patient symptoms, patient function, and quality-of-life. PROs have been used successfully to assess impairment in a clinical setting. Use of PROs to systematically quantify the patient experience provides valuable data to assist with clinical care; however, initiating use of PROs in clinical practice can be daunting. Here we provide suggestions for implementation of PROs and examples of opportunities to use PROs to tailor individual patient therapy to improve patient outcomes, patient-physician communication, and the quality of care for hematology/oncology patients. PMID:26637765

  19. Addressing challenges and needs in patient education targeting hardly reached patients with chronic diseases

    PubMed Central

    Varming, Annemarie Reinhardt; Torenholt, Rikke; Møller, Birgitte Lund; Vestergaard, Susanne; Engelund, Gitte

    2015-01-01

    Some patients do not benefit from participation in patient education due to reasons related to disease burden, literacy, and socioeconomic challenges. In this communication, we address more specifically both the challenges that these hardly reached patients face in relation to patient education programs and the challenges educators face when conducting patient education with hardly reached patients. We define principles for the format and content of dialogue tools to better support this patient group within the population of individuals with diabetes. PMID:25729695

  20. The soothing patients' anxiety 'SPA' experience.

    PubMed

    Harris, P

    2015-06-01

    This paper examines a blended approach to minimising patient anxiety levels prior to general anaesthesia for adult and paediatric patients with a learning disability by introducing reasonable adjustments and reasonable distractions. A therapeutic environment is created that promotes wellbeing; restrictive interventions are used only when there is potential for harm to the patient or others. The result can be excellent holistic individual patient care, the patient receiving 'added value' and a positive experience. PMID:26302589