96 research outputs found

    Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry

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    Many lysosomal storage diseases are characterized by an increased urinary excretion of glycoconjugates and oligosaccharides that are characteristic for the underlying enzymatic defect. Here, we have used capillary high-performance anion-exchange chromatography (HPAEC) hyphenated to mass spectrometry to analyze free oligosaccharides from urine samples of patients suffering from the lysosomal storage disorders fucosidosis, α-mannosidosis, GM1-gangliosidosis, GM2-gangliosidosis, and sialidosis. Glycan fingerprints were registered, and the patterns of accumulated oligosaccharides were found to reflect the specific blockages of the catabolic pathway. Our analytical approach allowed structural analysis of the excreted oligosaccharides and revealed several previously unpublished oligosaccharides. In conclusion, using online coupling of HPAEC with mass spectrometric detection, our study provides characteristic urinary oligosaccharide fingerprints with diagnostic potential for lysosomal storage disorders

    Do Neutrophils Play a Role in Establishing Liver Abscesses and Distant Metastases Caused by Klebsiella pneumoniae?

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    Serotype K1 Klebsiella pneumoniae is a major cause of liver abscesses and endophthalmitis. This study was designed to identify the role of neutrophils in the development of distant metastatic complications that were caused by serotype K1 K. pneumoniae. An in vitro cellular model was used to assess serum resistance and neutrophil-mediated killing. BALB/c mice were injected with neutrophils containing phagocytosed K. pneumoniae. Serotype K1 K. pneumoniae was significantly more resistant to serum killing, neutrophil-mediated phagocytosis and intra-cellular killing than non-K1 isolates (p<0.01). Electron microscopic examination had similar findings as in the bioassay findings. Intraperitoneal injection of neutrophils containing phagocytosed serotype K1 K. pneumoniae led to abscess formation in multiple sites including the subcutaneous tissue, lung, and liver, whereas no abscess formation was observed in mice injected with non-K1 isolates. The resistance of serotype K1 K. pneumoniae to complement- and neutrophil-mediated intracellular killing results in the dissemination of K. pneumoniae via the bloodstream. Escape from neutrophil intracellular killing may contribute to the dissemination and establishment of distant metastases. Thus, neutrophils play a role as a vehicle for helping K. pneumoniae and contributing to the establishment of liver abscess and distant metastatic complications

    Patients' experiences of peritoneal dialysis at home: a phenomenological approach

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    The aim of this study was to highlight the meaning of home dialysis as experienced by patients with chronic renal failure. The research design was influenced by Ricoeur´s phenomenology. Nineteen patients from a Brazilian public hospital were interviewed, from May to September 2009. Interviews were guided by the question: "Tell me about your experiences lived undergoing PD". Findings unveiled the patients' perception of the drastic changes in their existence, consequent to disease and treatment; and the perception of themselves in that process. The feeling of anguish, physical pain and deprivations were part of living that condition. They foresee an uncertain future, depending on the expertise of health care providers and the demands on support of significant others. Findings suggest that individual aspects of patients' experiences must be considered if health care providers are to facilitate positive health outcomes.El objetivo de este estudio fue comprender la experiencia de la diálisis peritoneal domiciliaria, a partir de la narración de los pacientes. El abordaje del estudio se inspiró en la fenomenología hermenéutica de Paul Ricoeur. Fueron entrevistados 19 pacientes en la unidad de hemodiálisis de un hospital público brasileño, de marzo a septiembre de 2009. Las entrevistas fueron orientadas por la pregunta: describa su experiencia en la diálisis peritoneal. Los resultados revelaron la percepción de los participantes sobre el significado de la enfermedad en sus vidas y las drásticas transformaciones personales sufridas en ese proceso. Sentimientos de angustia y dolor física fueron acompañados por importantes limitaciones personales y sociales, impuestas por el tratamiento. Ellos esperan un futuro desconocido, reconociendo su dependencia de la ayuda de los familiares y de los profesionales de la salud. Los resultados revelan las dificultades y la falta de perspectivas experimentadas por los pacientes en diálisis, demostrando el papel crucial que le cabe a los profesionales que los acompañan. Ayudarlos a desarrollar el autocuidado y maximizar su calidad de vida es una prioridad en la asistencia a esos pacientes.O objetivo deste estudo foi compreender a experiência da diálise peritoneal domiciliar, a partir da narrativa dos pacientes. A abordagem do estudo inspirou-se na fenomenologia hermenêutica de Paul Ricoeur. Foram entrevistados 19 pacientes na unidade de hemodiálise de um hospital público brasileiro, de março a setembro de 2009. As entrevistas foram orientadas pela questão: descreva sua experiência na diálise peritoneal. Os resultados desvelaram a percepção dos participantes sobre o significado da doença em suas vidas e as drásticas transformações pessoais sofridas nesse processo. Sentimentos de angústia e dor física foram acompanhados por importantes limitações pessoais e sociais, impostas pelo tratamento. Eles esperam por um futuro incerto, reconhecendo sua dependência da ajuda dos familiares e dos profissionais da saúde. Os resultados desvelam as dificuldades e a falta de perspectivas vividas pelos pacientes em diálise, demonstrando o papel crucial que cabe aos profissionais que os acompanham. Ajudá-los a desenvolver o autocuidado e maximizar sua qualidade de vida é prioridade na assistência a esses pacientes

    Synaptic integrative mechanisms for spatial cognition

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    Factors influencing quality of life in patients with benign primary brain tumors: prior to and following surgery

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    [[abstract]]GOALS: Few studies have repeatedly evaluated quality of life and potentially relevant factors in patients with benign primary brain tumor. The purpose of this study was to explore the relationship between the experience of the symptom distress, functional status, depression, and quality of life prior to surgery (T(1)) and 1 month post-discharge (T(2)). PATIENTS AND METHODS: This was a prospective cohort study including 58 patients with benign primary brain tumor in one teaching hospital in the Taipei area of Taiwan. The research instruments included the M.D. Anderson Symptom Inventory, the Functional Independence Measure scale, the Hospital Depression Scale, and the Functional Assessment of Cancer Therapy-Brain. RESULTS: Symptom distress (T(1): r = -0.90, p < 0.01; T(2): r = -0.52, p < 0.01), functional status (T(1): r = 0.56, p < 0.01), and depression (T(1): r = -0.71, p < 0.01) demonstrated a significant relationship with patients' quality of life. Multivariate analysis identified symptom distress (explained 80.2%, R (inc)(2) = 0.802, p = 0.001) and depression (explained 5.2%, R (inc)(2) = 0.052, p < 0.001) continued to have a significant independent influence on quality of life prior to surgery (T(1)) after controlling for key demographic and medical variables. Furthermore, only symptom distress (explained 27.1%, R (inc)(2) = 0.271, p = 0.001) continued to have a significant independent influence on quality of life at 1 month after discharge (T(2)). CONCLUSIONS: The study highlights the potential importance of a patient's symptom distress on quality of life prior to and following surgery. Health professionals should inquire about symptom distress over time. Specific interventions for symptoms may improve the symptom impact on quality of life. Additional studies should evaluate symptom distress on longer-term quality of life of patients with benign brain tumor
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