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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1997), S. 737-738 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Infection 3 (1975), S. 174-174 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. X-linked lymphoproliferative disease (XLP) is a rare inherited immunodeficiency to Epstein-Barr virus (EBV). The gene responsible for XLP has recently been identified as the four-exon SH2D1A gene encoding a 128-amino-acid protein that contains an SH2-domain. Functional studies indicate the SH2D1A protein acts as a regulator of at least two signal transduction pathways initiated by the cell surface molecules SLAM and 2B4, respectively, and possibly related to the host immune response to EBV infection. We have carried out a systematic mutation study of the SH2D1A gene in our series of 19 typical and 8 atypical XLP patients by polymerase chain reaction (PCR), reverse transcription/PCR, and sequencing, and have reconstructed the haplotypes of the patients. Four out of the 13 mutations detected are previously unreported. The identification of SH2D1A mutations in carriers from all three XLP families screened and the detection of mutations in two out of eight atypical patients indicates the usefulness of a DNA-based diagnosis for XLP disease.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of clinical microbiology & infectious diseases 15 (1996), S. 766-767 
    ISSN: 1435-4373
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Infection 11 (1983), S. 255-259 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Resultate der immunsuppressiven Behandlung (Kombination von Kortikosteroiden und Azathioprin) bei 104 Kindern mit HBsAg-positiver chronischer Hepatitis werden zusammengefaßt. Nach zwei bis sieben Behandlungsjahren war bei den meisten Fällen eine Genesung oder Besserung eingetreten. Diese Ergebnisse stehen im Widerspruch zur Ansicht anderer Autoren, die erwachsene Patienten behandelt haben. Eine Verschlechterung der biochemischen Parameter oder des histologischen Befundes wurde in einigen Fällen nach vorzeitigem Abbruch der Therapie beobachtet; bei Wiedereinsetzen der Behandlung trat erneut eine Besserung ein. Schwerwiegende Nebenwirkungen wurden bei dieser Kombinationstherapie nicht beobachtet.
    Notes: Summary The results of immunosuppressive treatment (a corticosteroid-azathioprine combination) in 104 children suffering from chronic HBsAg-positive hepatitis are reviewed. A recovery and/or improvement of the majority of the cases treated was observed after two to seven years of therapy, in contradiction to the opinion of other authors who treated adult patients. A biochemical and/or bioptic deterioration was observed in some cases in which treatment was interrupted too soon; this was followed by an improvement when treatment was re-initiated. No serious side-effects were observed with the combination treatment.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 36 (1991), S. 1143-1146 
    ISSN: 1573-2568
    Keywords: hepatocellular carcinoma ; chronic hepatitis ; HBV DNA ; viral DNA integration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a series of 325 HBV chronically infected children observed over an 18-year period, three developed HCC. These three children were born in southern Italy, a region characterized by a high endemic HBV infection rate; each had been infected perinatally, developed an acute hepatitis, and became a chronic carrier. Two of the three with cirrhosis were HBsAg positive at the time their HCC was detected. The remaining case had seroconverted to HBsAb but HBV-DNA integration could be demonstrated in the absence of cirrhosis; moreover HBV antigens were not expressed in the tissue of this case. The interval between HBV infection and HCC appearance in these three cases ranged from six to 11 years. A similarity between these three Italian cases and the majority of HCC, arising in chronically infected children in the Far East is noted.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Infection 3 (1975), S. 78-82 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurden Lymphozyten von 22 mit chronischer Hepatitis befallenen Kindern mit 32 gesunden gleichaltrigen Kindern im Hinblick auf die DNA-Stimulierung, die E-Rosetten-Bildung und das Membran-Immunglobulin verglichen. Es ergab sich folgendes: Die Kurve der PHA-Stimulierung war nach links verschoben (niedrigere PHA-Dosis), die DNA-Synthese war bei den getesteten PHA-Dosierungen signifikant erhöht. Das Plasma von den Hepatitiskranken erwies sich weder als hemmend noch als stimulierend. Der Prozentsatz von E-Rosetten-bildenden Zellen war abgesunken, aber die Gesamtzellenzahl war normal. Die mittlere Prozentzahl von Ig-tragenden Zellen war normal, jedoch schwankten die einzelnen Werte in einem breiten Bereich. Es ist zu vermuten, wenigstens bei chronischen Erkrankungen, daß eine gesteigerte PHA-Reaktion auftritt, sowie eine prozentuale Zunahme von Zellen, die weder als B-noch als T-Zellen bei der Markierung in Erscheinung traten.
    Notes: Summary Lymphocytes from 22 children affected by chronic hepatitis were studied and a comparison was made with a control group of 32 healthy children in the same age range with respect to PHA dose-response curves, E-rosette formation and membrane immunoglobulin (Ig). From the results it appeared that PHA dose-response curves were displaced to the left (low PHA dose), DNA synthesis was significantly higher for all PHA dosages tested, and that the plasma of chronic hepatitis patients acted neither as inhibitor nor stimulator. The percentage of E-rosette-forming cells decreased, but the total number was normal. The mean percentage of Ig-bearing cells was normal but the single values were spaced over a wide range. It can be assumed that, at least in the chronic stage, there is an enhanced response to PHA and an increased percentage of cells without markers either for T or for B cells.
    Type of Medium: Electronic Resource
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