78 research outputs found

    Isotropic apparent diffusion coefficient mapping of postnatal cerebral development

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    Diffusion-weighted imaging (DWI) allows us to image the motion of tissue water. This has been used to demonstrate acute ischaemia. Diffusion imaging is also sensitive to water movement along neuronal tracts. Our objective was to map brain maturation in vivo using maps of apparent diffusion coefficient (ADC). We studied 22children without neurological disease aged between 2 and 720days. MRI was performed at 1.5tesla. Multislice single-shot echoplanar DWI was performed at b 0 and 1000s/mm2. ADC maps were generated automatically and measurements were performed in the basal ganglia, frontal and temporal white matter and the pons. There was a decrease over time in water diffusion in the areas examined, most marked in the frontal (0.887-1.898×10-3mm2/s) and temporal (1.077-1.748×10-3mm2/s)lobes. There was little change, after an initial decrease, in the basal ganglia (0.690-1.336×10-3mm2/s). There was a difference in water diffusion between the anterior (0.687-1.581×10-3mm2/s) and posterior (0.533-1.393×10-3mm2/s) pons. These changes correlate well with those observed in progressive myelination: the increased water content probably reflects incomplete myelination and the decrease with time in water motion reflects the increase in myelinated brai

    Aerosolized amikacin for treatment of pulmonary Mycobacterium avium infections: an observational case series

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    BACKGROUND: Current systemic therapy for nontuberculous mycobacterial pulmonary infection is limited by poor clinical response rates, drug toxicities and side effects. The addition of aerosolized amikacin to standard oral therapy for nontuberculous mycobacterial pulmonary infection may improve treatment efficacy without producing systemic toxicity. This study was undertaken to assess the safety, tolerability and preliminary clinical benefits of the addition of aerosolized amikacin to a standard macrolide-based oral treatment regimen. CASE PRESENTATIONS: Six HIV-negative patients with Mycobacterium avium intracellulare pulmonary infections who had failed standard therapy were administered aerosolized amikacin at 15 mg/kg daily in addition to standard multi-drug macrolide-based oral therapy. Patients were monitored clinically and serial sputum cultures were obtained to assess response to therapy. Symptomatic improvement with radiographic stabilization and eradication of mycobacterium from sputum were considered markers of success. Of the six patients treated with daily aerosolized amikacin, five responded to therapy. All of the responders achieved symptomatic improvement and four were sputum culture negative after 6 months of therapy. Two patients became re-infected with Mycobacterium avium intracellulare after 7 and 21 months of treatment. One of the responders who was initially diagnosed with Mycobacterium avium intracellulare became sputum culture positive for Mycobacterium chelonae resistant to amikacin after being on intermittent therapy for 4 years. One patient had progressive respiratory failure and died despite additional therapy. There was no evidence of nephrotoxicity or ototoxicity associated with therapy. CONCLUSION: Aerosolized delivery of amikacin is a promising adjunct to standard therapy for pulmonary nontuberculous mycobacterial infections. Larger prospective trials are needed to define its optimal role in therapy of this disease

    Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations

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    Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease affecting multiple body systems with wide variability in presentation. In 2013, Pediatric Neurology published articles outlining updated diagnostic criteria and recommendations for surveillance and management of disease manifestations. Advances in knowledge and approvals of new therapies necessitated a revision of those criteria and recommendations. Methods: Chairs and working group cochairs from the 2012 International TSC Consensus Group were invited to meet face-to-face over two days at the 2018 World TSC Conference on July 25 and 26 in Dallas, TX, USA. Before the meeting, working group cochairs worked with group members via e-mail and telephone to (1) review TSC literature since the 2013 publication, (2) confirm or amend prior recommendations, and (3) provide new recommendations as required. Results: Only two changes were made to clinical diagnostic criteria reported in 2013: “multiple cortical tubers and/or radial migration lines” replaced the more general term “cortical dysplasias,” and sclerotic bone lesions were reinstated as a minor criterion. Genetic diagnostic criteria were reaffirmed, including highlighting recent findings that some individuals with TSC are genetically mosaic for variants in TSC1 or TSC2. Changes to surveillance and management criteria largely reflected increased emphasis on early screening for electroencephalographic abnormalities, enhanced surveillance and management of TSC-associated neuropsychiatric disorders, and new medication approvals. Conclusions: Updated TSC diagnostic criteria and surveillance and management recommendations presented here should provide an improved framework for optimal care of those living with TSC and their families

    Updated international tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations

    Get PDF
    Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease affecting multiple body systems with wide variability in presentation. In 2013, Pediatric Neurology published articles outlining updated diagnostic criteria and recommendations for surveillance and management of disease manifestations. Advances in knowledge and approvals of new therapies necessitated a revision of those criteria and recommendations. Methods Chairs and working group cochairs from the 2012 International TSC Consensus Group were invited to meet face-to-face over two days at the 2018 World TSC Conference on July 25 and 26 in Dallas, TX, USA. Before the meeting, working group cochairs worked with group members via e-mail and telephone to (1) review TSC literature since the 2013 publication, (2) confirm or amend prior recommendations, and (3) provide new recommendations as required. Results Only two changes were made to clinical diagnostic criteria reported in 2013: “multiple cortical tubers and/or radial migration lines” replaced the more general term “cortical dysplasias,” and sclerotic bone lesions were reinstated as a minor criterion. Genetic diagnostic criteria were reaffirmed, including highlighting recent findings that some individuals with TSC are genetically mosaic for variants in TSC1 or TSC2. Changes to surveillance and management criteria largely reflected increased emphasis on early screening for electroencephalographic abnormalities, enhanced surveillance and management of TSC-associated neuropsychiatric disorders, and new medication approvals. Conclusions Updated TSC diagnostic criteria and surveillance and management recommendations presented here should provide an improved framework for optimal care of those living with TSC and their families

    Sulphite content of atmospheric aerosol particles.

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    The content of sulphite in atmospheric aerosol particles is investigated by filter and cascade impactor measurents. The role of atmosheric trace gases and meteorological conditions for the formation of sulphite in particles is discussed

    The input of inorganic ions to spruce forests by dry deposition of aerosol particles.

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    In order to estimate the dry deposition of the major inorganic ions to two different spruce standsthe input parameters of a model for determining the deposition velocity were continuously measured. Size specific samples of particulate matter were collected at the same sites with five-stage Berner impactors. The dry deposition flux F was calculated by multiplying these concentrations by the deposition velocity specific to each measuring interval and size class

    Short term variations of size distributions and concentrations ofatmospheric aerosol particles.

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    With a Mobile Aerosol Spectrometer variations of size distributions during differentmeteorological events were measured. The correlation between aerosol data, Nitrogene Oxide concentrations and meteorological parameters was studied

    Intercomparison of different aethalometers with an absorption technique: Laboratory calibrations and field measurements.

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    The prototype of a new aethalometer was calibrated and tested in the laboratory by applying graphite, salt and iron oxide aerosol particles. It turned out that the response of the aethalometer was critical to the ratio of the mass of absorbing and non-absorbing materials. The aethalometer data from field measurements were compared with results gained by an optical absorption technique (Hanel's method). The correlations showed wide scattering, presumably due to different compositions of atmospheric particles. During field measurements the prototype was also compared with a commercial aethalometer of the 'Hansen-type'. The measured soot concentrations differed by about 50% on average. The results of the commercial aethalometer and the absorption technique agreed very well.The prototype of a new aethalometer was calibrated and tested in the laboratory by applying graphite, salt and iron oxide acrosol particles. It turned out that the response of the acthalometer was critical to the ratio of the mass of absorbing and non-absorbing materials. The aethalometer data from field measurements were compared with results gained by an optical absorption technique (Hanel's method). The correlations showed wide scattering, presumably due to different compositions of atmospheric particles. During field measurements the prototype was also compared with a commercial aethalometer of the 'Hansen-type'. The measured soot concentrations differed by about 50% on average. The results of the commercial aethalometer and the absorption technique agreed very well
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