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  • 11
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 21 (1998), S. 604-612 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cloning of the gene defective in the X-linked neurodegenerative disorder Menkes disease led to a cascade of new findings. Besides giving a better understanding of the intracellular copper homeostasis, these findings had important consequences from a clinical point of view. Today the underlying genetic defect has been described in several patients affected by one of the three hereditary disorders of copper metabolism: Menkes disease, occipital horn syndrome and Wilson disease. In this review we discuss mainly Menkes disease and the impact of the recent findings on the diagnosis of this disorder.
    Type of Medium: Electronic Resource
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  • 12
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 267-270 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 13
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 6 (1983), S. 59-62 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Increased64Cu uptake into cultured cells is a biochemical marker for mutant cells in Menkes' disease (McKusick 30940). Using this marker selective prenatal diagnosis has been carried out in more than 80 at-risk pregnancies. The64Cu uptake into cultures from affected male fetuses is, however, negatively correlated to the fetal age at amniocentesis. After the 18th week of gestation the risk of false negatives is significant. Using copper uptake into uncloned cultures, a number of obligate and possible carriers showed significantly increased values, but the range of values of obligate carriers considerably overlapped those of the normal controls. All values of normal controls were within a limited range and values above the upper limit in females at risk must, therefore, be caused by mutant cells and establish the carrier diagnosis. However, the extreme skewing of the distribution towards normal values in obligate carriers indicates a strong selection against the mutant cell type and this will hamper the detection of all female carriers in risk families. C-banding heteromorphism of the X-chromosome provides a supplementary carrier detection method. Linkage analysis in five Danish families demonstrated a close physical relationship between the gene for Menkes' disease and the centromere region. By comparative gene mapping (mouse/man) the most likely localization of the gene for Menkes' disease can be suggested to be in band q13 on the long arm of the human X-chromosome. This regional assignment facilitates the choice of appropriate X-specific DNA probes in search for linkage at the DNA level.
    Type of Medium: Electronic Resource
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  • 14
    Electronic Resource
    Electronic Resource
    Springer
    Intensivmedizin und Notfallmedizin 36 (1999), S. 635-639 
    ISSN: 1435-1420
    Keywords: Key words Acute renal failure ; uremic encephalopathy ; emergency medicine ; benign prostatic hypertrophy ; Schlüsselwörter Akutes Nierenversagen ; urämische Enzephalopathie ; Notfallmedizin ; benigne Prostatahypertrophie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Unklare Bewußtseinsstörungen sind ein häufiger Alarmierungsgrund für den Notarzt. So wurde auch im vorliegenden Fall eines 71-jährigen Patienten mit einer plötzlichen Bewußtseinstrübung der Notarzt gerufen. Als Auslöser der Bewußtseinsstörung fand sich eine urämische Enzephalopathie bei akutem Nierenversagen. Der Patient wurde auf die internistische Intensivstation aufgenommen und in den folgenden zwei Tagen dreimal hämodialysiert. Danach stabilisierte sich die Nierenfunktion. Als Ursache des renalen Versagens fand sich eine Hydronephrose bei Prostataadenom, die drei Wochen später transurethral reseziert wurde. Die Kasuistik zeigt, daß bei unklaren Bewußtseinsstörungen im Notarztdienst auch seltene metabolische Krankheitsbilder differentialdiagnostisch zu berücksichtigen sind.
    Notes: Summary Neurocognitive disorders are often the reason to call for an emergency physician. We report on a 71 year old male patient who showed a sudden sensorial clouding caused by an uremic encephalopathy. The patient was admitted to the intensive car unit. During the following two days he underwent three emergency hemodialysis sessions until renal function recovered. Further investigations revealed as hydronephrosis secondary to a benign prostatic hypertrophy as the cause of acute renal failure
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 12 (1989), S. 207-214 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 105 patients with Menkes disease have been diagnosed from64Cu-uptake studies in fibroblasts. These results are presented together with chase results following removal of64Cu from the medium for 16 Menkes patients. Second-trimester prenatal diagnosis has been performed in 80 pregnancies with male karyotype. These64Cu-uptake results show some overlap between the upper end of the normal range and the lower end of the Menkes range. Results are presented to show that a combination of64Cu-uptake and chase results offers a better diagnostic potential than64Cu-uptakeper se. Chorionic villus copper values from 53 first-trimester prenatal diagnoses are presented. Maternal deciduum from some of these pregnancies contain similar high amounts of copper as found in the chorionic villus samples from affected fetuses.64Cu-uptake in cultured chorionic villi from affected fetuses and unaffected fetuses is not discriminatory. Chase results seem however to offer a better diagnostic potential.
    Type of Medium: Electronic Resource
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  • 16
    Electronic Resource
    Electronic Resource
    Springer
    European journal of plant pathology 101 (1995), S. 221-224 
    ISSN: 1573-8469
    Keywords: chickpea stunt
    Source: Springer Online Journal Archives 1860-2000
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Notes: Abstract Yield losses caused by chickpea chlorotic dwarf virus in chickpea were estimated by comparing uninfected and infected plants in the field at two locations in India. When infection was before flowering, yield losses of individual plants amounted to nearly 100% in the three cultivars studies. Plants that became infected during flowering had yield losses of 75–90%. Percentage of crop loss is likely to equal percentage of disease incidence, since plant densities in farmers' fields are probably too low to allow uninfected plants around infected ones to compensate the yield losses of infected plants.
    Type of Medium: Electronic Resource
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  • 17
    Electronic Resource
    Electronic Resource
    Springer
    European journal of plant pathology 95 (1989), S. 123-127 
    ISSN: 1573-8469
    Keywords: Arachis hypogaea ; peanut mottle virus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Description / Table of Contents: Samenvatting Tijdens een inspectie in 1986 en 1987 in Zuid Sulawesi, West en Oost Java en West Sumatra werd in aardnoot (Arachis hypogaea) veelvuldig een ziekte aangetroffen, die opviel door donkergroene vlekkerigheid. De vlekken waren onregelmatig over de bladeren verspreid of kwamen voornamelijk voor rond de nerven. Soms waren de vlekken omgeven door een lichtgroene ring. Op grond van symptomen op toetsplanten, serologische verwantschap en elektronenmicroscopische eigenschappen werd geconcludeerd, dat deze ziekte veroorzaakt wordt door het ‘peanut stripe virus’. Dit virus is voor het eerst beschreven in 1984 in Amerika. Dit is de eerste officiële melding van het virus in Indonesië. Enkele eerder beschreven potyvirussen van aardnoot in Indonesië, ‘groundnut mottle-y’ en ‘peanut mottle virus’ (PMV) zijn waarschijnlijk ook PStV. De gelijkenis van de door PStV and ‘peanut mottle virus’ veroorzaakte symptomen heeft ervoor gezorgd dat de vlekkerigheid op aardnoot in Indonesië jarenlang aan PMV werd toegeschreven. Aangezien PStV veel schade veroorzaakt, wordt verder onderzoek gedaan aan de epidemiologie van dit virus, aan het oogstverlies en aan het toetsen van aardnootlijnen op resistentie.
    Notes: Abstract During a survey of groundnut in 1986 and 1987 in South Sulawesi, West and East Java and West Sumatra a disease was frequently observed which is characterized by the presence of green blotches. These blotches were either randomly distributed over the entire leaflets or concentrated along the veins; sometimes they were surrounded by light green rings. On the basis of test plant studies, serology and electron microscopy it was concluded that the causal agent of the disease is peanut stripe virus (PStV). This virus has been described for the first time in the USA in 1984. Some potyviruses described earlier in Indonesia, viz. groundnut mottle-y and peanut mottle virus (PMV) probably are also PStV. The similarity of symptoms caused by PStV and peanut mottle virus was the reason why blotching on groundnut in Indonesia was ascribed for many years to PMV. As PStV causes severe yield losses, further research is being done on the epidemiology of the virus, on yield loss assesment and on testing breeding lines of groundnut for resistance.
    Type of Medium: Electronic Resource
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  • 18
    Electronic Resource
    Electronic Resource
    Springer
    European journal of plant pathology 92 (1986), S. 21-32 
    ISSN: 1573-8469
    Keywords: fungicide resistance ; fenpropimorph ; propiconazole ; triadimefon ; Erysiphe graminis f.sp.tritici
    Source: Springer Online Journal Archives 1860-2000
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Description / Table of Contents: Samenvatting Fungiciden die de ergosterolbiosynthese remmen worden in Nederland sinds 1978 gebruikt bij de bestrijding van tarwemeeldauw (Erysiphe graminis f. sp.tritici) Meeldauwpopulaties werden getoetst op hun variatie in gevoeligheid voor triadimefon van 1982 tot 1984. In 1982 bleken isolaten afkomstig uit de provincie Limburg, waar voordien triazolen werden toegepast, minder gevoelig te zijn voor triadimefon dan isolaten uit de provincies Gelderland en Noord-Brabant, waar nog geen triazolen werden gebruikt. In de daaropvolgende jaren werden isolaten met een verminderde gevoeligheid ook in laatstgenoemde provincies en in andere delen van het land gevonden. Deze uitbreiding is gecorreleerd met een toename in het gebruik van triazolen vanaf 1983 in areaal en frequentie. De afname in gevoeligheid kan geheel of gedeeltelijk verantwoordelijk zijn voor de verminderde meeldauwwerking van triazolen die gedurende deze jaren werd waargenomen. Kruisgevoeligheid werd vastgesteld voor de triazolen triadimefon en propiconazool, maar niet voor triazolen en het morfoline-derivaat fenpropimorf. De werking van dit middel was tegen alle isolaten uit Limburg die in 1984 werden getoetst, gelijk. De meeldauwwerking van fenpropimorf, dat in 1983 werd geïntroduceerd, was normaal. Aanbevolen wordt om verdere resistentie-ontwikkeling tegen te gaan door afwisselend gebruik van fenpropimorf vroeg in het groeiseizoen (mei) en een triazool aan het eind (juni-juli).
    Notes: Abstract Fungicides which inhibit ergosterol biosynthesis have been in use for control of wheat powdery mildew (Erysiphe graminis f. sp.tritici) in the Netherlands since 1978. Mildew populations were tested for their variation in sensitivity to triadimefon from 1982 to 1984. In 1982 isolates from the province Limburg, with a triazole spray-regime history, were less sensitive to triadimefon than isolates from the provinces Gelderland and Noord-Brabant, where triazoles had not been used. In the following years isolates with reduced sensitivity were also detected in the latter provinces and other parts of the country. This spread correlates with the increased use of triazoles, both in frequency and space, from 1983 onwards. The reduced sensitivity can as a whole or in part be responsible for the decline in field performance of triazoles, observed during these years. Cross-sensitivity to the triazoles triadimefon and propiconazole was established, but not to triazoles and the morpholine fungicide fenpropimorph. Effectiveness of the latter compound was similar to all isolates from Limburg tested in 1984. Field performance of fenpropimorph, introduced in 1983, appeared to be normal. It is recommended to counteract further development of resistance by sequential use of fenpropimorph early in the season (May) and triazoles at the end (June–July).
    Type of Medium: Electronic Resource
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