2,606 research outputs found
CGD-UK_sources
No full textCGD-UK_sources contains details of the sources from which CGD-UK data have been compiled</p
CGD-UK_unsorted _data_tables
No full textCGD-UK_unsorted_CatalogueGeothermalData1984_Table_1.csv: CSV file containing a copy of Table 1 of UKGC-3. This table has been read from scanned documents using automated data-recognition software. It lists selected temperature measurements and associated metadata.
CGD-UK_unsorted_CatalogueGeothermalData1984_Table_2.csv: CSV file containing a machine-read copy of Table 2 of UKGC-2. This table has been read from a scanned document using automated data-recognition software. It lists estimates of heat flow and associated metadata.
CGD-UK_unsorted_CatalogueGeothermalData1984_Table_3.csv: CSV file containing a machine-read copy of Table 3 of UKGC-3. This table has been read from a scanned document using automated data-recognition software. It lists selected geochemical measurements and associated metadata.
CGD-UK_UKCS_CGG_GeothermalDatabase.csv: CSV file containing measurements of temperature from offshore boreholes on the UK Continental Shelf. This database has been compiled and generously provided by CGG. This currently includes temperature measurements from 2400 unique well locations on the UKCS.
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Chronic granulomatous disease: the European experience.
No full textCGD is an immunodeficiency caused by deletions or mutations in genes that encode subunits of the leukocyte NADPH oxidase complex. Normally, assembly of the NADPH oxidase complex in phagosomes of certain phagocytic cells leads to a "respiratory burst", essential for the clearance of phagocytosed micro-organisms. CGD patients lack this mechanism, which leads to life-threatening infections and granuloma formation. However, a clear picture of the clinical course of CGD is hampered by its low prevalence (approximately 1:250,000). Therefore, extensive clinical data from 429 European patients were collected and analyzed. Of these patients 351 were males and 78 were females. X-linked (XL) CGD (gp91(phox) deficient) accounted for 67% of the cases, autosomal recessive (AR) inheritance for 33%. AR-CGD was diagnosed later in life, and the mean survival time was significantly better in AR patients (49.6 years) than in XL CGD (37.8 years), suggesting a milder disease course in AR patients. The disease manifested itself most frequently in the lungs (66% of patients), skin (53%), lymph nodes (50%), gastrointestinal tract (48%) and liver (32%). The most frequently cultured micro-organisms per episode were Staphylococcus aureus (30%), Aspergillus spp. (26%), and Salmonella spp. (16%). Surprisingly, Pseudomonas spp. (2%) and Burkholderia cepacia (<1%) were found only sporadically. Lesions induced by inoculation with BCG occurred in 8% of the patients. Only 71% of the patients received antibiotic maintenance therapy, and 53% antifungal prophylaxis. 33% were treated with gamma-interferon. 24 patients (6%) had received a stem cell transplantation. The most prominent reason of death was pneumonia and pulmonary abscess (18/84 cases), septicemia (16/84) and brain abscess (4/84). These data provide further insight in the clinical course of CGD in Europe and hopefully can help to increase awareness and optimize the treatment of these patients
Review Article Children with Chronic Granulomatous Disease
No full textPatients with CGD are "experiments of nature." An abnormality of genetic coding of small part of a protein (cytochrome b) in the membrane of phagocytic cells results in abnormal oxidative metabolism of these cells. The metabolic defect is critical for production of reactive oxygen radicals, which are necessary for efficient intracellular killing of catalase-positive bacterial and fungal species within phagocytic vacuoles. Patients with CGD suffer recurrent severe and often lifethreatening infections with these same species of bacteria and fungi. Thus clinical evidence is provided for the importance of a normal oxidative response of phagocytic cells during the engulfment process for normal host defense against bacteria. Investigators, intrigued by this remarkable biochemical clinical correlation, have studied human granulocytes with the tools of modern molecular genetics. The abnormal gene has been located and defective-gene products have been identified in CGD patients. This knowledge has provided a basis for therapy of CGD patients with human recombinant interferon gamma, an immunomodulator which stimulates NADPH-oxidase activity in the abnormal granulocytes. Other treatment and replacement modalities are anticipated but most importantly these CGD patients have provided insights into the usually hidden mysteries of nature. We are very grateful to these patients as our teachers
CGD-UK_thermal_conductivity_individual_measurements
No full textIndividual csv for borehole thermal conductivity measurements.
File named using BGS Single Onshore Borehole Identifier reference</p
CGD-UK_startigraphy_lithologies
No full textCSV files with names of the form XXX_ukogl_well_tops.csv, where XXX is the unique identifier describing each location. Each file contains stratigraphic and lithographic information from the UK Onshore Geophysical Library (UKOGL) for the corresponding locations.</p
CGD-UK_thermal_conductivity_compilations
No full textCSV files with names of the form XXX_conds.csv, where XXX is the unique identifier describing each location. These files contain thermal conductivity measurements made at the corresponding locations.</p
CGD-UK_temperature_individual_measurements
No full textIndividual csv for borehole temperature measurements.
File named using BGS Single Onshore Borehole Identifier reference</p
CGD-UK_master_table
No full textMaster table providing an index of individual borehole records for temperature and thermal conductivity data. </p
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