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  • 1
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Congenital MHC class II deficiency or bare lymphocyte syndrome (BLS; McKusick 209920) is caused by defects in trans-acting regulatory factors that control MHC class II expression and is therefore a disease of gene regulation. There are at least four complementation groups and the genetic and molecular dissection of this rare disease has contributed considerably to our current understanding of the molecular mechanisms governing MHC class II expression. Identification of the gene that is defective in BLS complementation group A, CIITA (MHC class II transactivator), has led to the discovery that CIITA acts as a master control factor of MHC class II expression. We have identified the CIITA mutations in a second patient from BLS group A. Two novel mutations abolish CIITA function, as shown by transfection experiments. Molecular analysis of these two novel mutations, together with the one described earlier in the first patient, is informative in terms of CIITA structure-function relationships.
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  • 2
    ISSN: 1433-0474
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Der Chirurg 69 (1998), S. 298-300 
    ISSN: 1433-0385
    Keywords: Key words: Adrenal pseudocyst ; Adrenal cyst ; Operative therapy. ; Schlüsselwörter: Adrenale Pseudocysten ; Nebennierencysten ; operative Therapie.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung. Wir berichten über einen 64 jährigen Patienten mit einer monströsen adrenalen Pseudocyste. Dabei handelt es sich um seltene cystische Läsionen der Nebenniere, deren genaue Ätiologie noch unklar ist. Neuere Untersuchungen deuten jedoch darauf hin, daß ein Großteil der adrenalen Pseudocysten vasculärer Genese ist. Differentialdiagnostisch müssen alle cystischen Raumforderungen im Oberbauch in Erwägung gezogen werden. Die Therapie der Wahl ist die operative Entfernung.
    Notes: Summary. We report on a 64-year-old patient with a huge adrenal pseudocyst. These are rare cystic lesions of the adrenal gland with an unclear etiology. Recent studies suggest that the majority of adrenal pseudocysts are of vascular origin. All cystic masses in the epigastric region have to be considered in the differential diagnosis. The treatment of choice is surgical removal.
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  • 4
    ISSN: 1432-8798
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary.  Differences in early events during entry and integration of HIV-1into peripheral blood mononuclear cells (PBMC) might contribute to theabsence of AIDS-like disease in chimpanzees as compared to humans. Toaddress this question, we first tested the in vitro susceptibility of humanand chimpanzee PBMC for infection with the two HIV-1 isolates III B andRF. The results of these studies revealed that chimpanzee PBMC had aslightly lower capability to support the growth of HIV-1 as compared tohuman PBMC. This was accompanied by a delayed accumulation of proviral HIV-1 DNA in cultures of HIV-1-infected chimpanzee PBMC. However, nodifferences between cells of the two species were observed when very earlyevents of HIV-1 infection were studied. Shortly (20 h) after infectionchimpanzee and human cells harbored similar amounts of proviral HIV-1 DNA andPBMC of both species behaved comparably with respect to pre-integrationlatency (i.e. the ability to activate extrachromosomal HIV-1 intermediatesin HIV-1 infected quiescent cells at various times after infection). Theseresults strongly suggest that the absence of AIDS-like disease inchimpanzees cannot be correlated with defects in early events of the HIV-1replicative cycle.}
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Der Gynäkologe 29 (1996), S. 677-683 
    ISSN: 1433-0393
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung Im Vordergrund der Symptomatik der Rektozele aus chirurgischer Sicht steht die Defäkationsstörung. Ventrale Ballonierung und digitale Unterstützung des Damms bei der Defäkation sowie das Gefühl der inkompletten Entleerung sind die Leitsymptome. Die ergänzende Diagnostik soll Transitstörungen oder einen Anismus ausschließen und gleichzeitig bestehende Genital- oder Blasensenkungen erfassen. Die Entwicklung der chirurgischen Verfahren, deren Ergebnisse und Modifikationen wird beschrieben. Therapeutisch erscheint die Defäkationsproblematik durch Stabilisierung und Verstärkung des Septum rectovaginale und des Perinealkörpers bei gleichzeitiger Resektion überschüssiger Rektummukosa und Längsraffung der ausgeweiteten Rektumwand auf peranalem Wege am besten erreichbar. Gute bis sehr gute Ergebnisse werden in etwa 80 % bei vergleichsweise geringen Komplikationsraten beschrieben. Die Behauptung, daß die Defäkationsstörung nur durch peranale Korrektur sicher zu behandeln sei, wird allerdings nicht durch prospektiv vergleichende Studien gestützt. Studien zum Vergleich der peranalen mit der transvaginalen Korrektur und deren funktioneller Spätergebnisse sind daher zu fordern. Nur die symptomatische Rektozele sollte operativ behandelt werden.
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  • 6
    ISSN: 1432-0584
    Keywords: Key words CLL ; Spontaneous remission ; Immunoglobulin deficiency ; Secondary neoplasia ; Hodgkin's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We present a 71-year-old patient with chronic lymphocytic leukemia diagnosed 27 years ago. Initially, the disease was staged as Rai II and the patient suffered from secondary immunoglobulin deficiency. Nevertheless, no treatment was necessary at that time. Because of disease progression a single course of chemotherapy was given in 1984. During the following year there was a constant decline of the WBC, accompanied by normalization of the immunoglobulins; both have remained stable ever since that time. However, there was still residual bone marrow infiltration, indicating persisting CLL. In 1993 cervical lymphadenopathy occurred with acute onset. A diagnostic lymphadenectomy revealed Hodgkin's disease of the nodular-sclerosing subtype. The patient was staged as II-III according to the Ann Arbor Classification and underwent radiation therapy. Cytogenetic examination of the bone marrow revealed a normal karyotype with an inversion of chromosome 9. This case demonstrates the rare coincidence of two lymphoproliferative disorders in the same patient. The clinical course and the immunologic findings of this patient are presented, together with a review of the literature.
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  • 7
    ISSN: 1432-0584
    Keywords: CLL ; Spontaneous remission ; Immunoglobulin deficiency ; Secondary neoplasia ; Hodgkin's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a 71-year-old patient with chronic lymphocytic leukemia diagnosed 27 years ago. Initially, the disease was staged as Rai II and the patient suffered from secondary immunoglobulin deficiency. Nevertheless, no treatment was necessary at that time. Because of disease progression a single course of chemotherapy was given in 1984. During the following year there was a constant decline of the WBC, accompanied by normalization of the immunoglobulins; both have remained stable ever since that time. However, there was still residual bone marrow infiltration, indicating persisting CLL. In 1993 cervical lymphadenopathy occurred with acute onset. A diagnostic lymphadenectomy revealed Hodgkin's disease of the nodular-sclerosing subtype. The patient was staged as II–III according to the Ann Arbor Classification and underwent radiation therapy. Cytogenetic examination of the bone marrow revealed a normal karyotype with an inversion of chromosome 9. This case demonstrates the rare coincidence of two lymphoproliferative disorders in the same patient. The clinical course and the immunologic findings of this patient are presented, together with a review of the literature.
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  • 8
    ISSN: 1432-0843
    Keywords: Key words Alkylphosphocholines ; Erucylphosphocholine (ErPC) ; Biodistribution ; Pharmacokinetics ; Chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical use of alkylphosphocholines (APC) in cancer patients is restricted because of the high gastrointestinal toxicity and the need for oral administration. Therefore we evaluated the clinical pharmacology of erucylphosphocholine (ErPC), the first derivative of the APC family suitable for intravenous administration with strong antineoplastic activity, in vitro and in vivo in rats. The pharmacokinetic parameters after a single intravenous dose of 40 mg/kg were calculated using a two-compartment model: Cmax= 1.6 ± 0.3 μmol/ml, T1/2α= 0.18 ± 0.09 h, T1/2β= 3.3 ± 0.88 h, clearance = 9.7 ± 1.2 ml/h, AUC = 2.5 ± 0.3 μmol/ml per h and Vss = 40.4 ± 7.9 ml. Biodistribution studies were performed after repeated ErPC administration at different doses. Intravenous injections of 20 mg/kg given at intervals of 48 h for up to 4 weeks were well tolerated. Neither clinical evaluation nor laboratory parameters (haematology and clinical chemistry) revealed toxic side effects. In contrast, higher doses of ErPC (40 mg/kg per 48 h) led to weight loss. After 2 and 4 weeks of therapy with 20 mg/kg per 48 h a high ErPC accumulation was found in the adrenal glands, small intestine and brain. The brain to serum concentration ratios averaged 2.1 after 2 weeks and 4.5 after 4 weeks. Significant leucocytosis and thrombocytosis were observed after 4 weeks of ErPC treatment. The findings suggest that ErPC is a suitable candidate for clinical trials. In particular, owing to the high accumulation in brain tissue, ErPC is a potential agent for chemotherapy against malignant brain tumours.
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  • 9
    ISSN: 1432-0843
    Keywords: Key words Alkylphosphocholines ; HL-60 cells ; U-937 cells ; K-562 cells ; Apoptosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Alkylphosphocholines (APC) represent a new group of ether-lipid-related compounds with remarkable activity against transformed cells in vitro and good tolerability in vivo. Their mechanism of action remains unknown. The aim of the present study was to investigate the effects of a series of APC on three human leukemic cell lines: K-562, HL-60, and U-937. The tetrazolium dye-reduction (MTT) assay and cell counting were used to determine the cytotoxicity of the APC used. DNA gel electrophoresis and enzyme-linked immunosorbent assay (ELISA) detection of oligonucleosomes were performed to identify and quantify DNA fragmentation. Electron and phase-contrast microscopy were used to detect morphologic changes specific for programmed cell death. HL-60 and U-937 cells were found to be sensitive, but K-562 cells were relatively resistant to APC exposure. APC with long alkyl chains exerted stronger cytotoxicity than did those with short alkyl chains. DNA fragmentation was found after treatment with APC in HL-60 and U-937 cells but not in K-562 cells. In HL-60 cells the increase in mono- and oligonucleosome formation as measured by ELISA was correlated with the length of the alkyl chains at 14 h of exposure to APC but plateaued at 20 h. The morphologic alterations in HL-60 and U-937 cell lines, such as cell shrinkage, chromatin condensation, and formation of apoptotic bodies, confirmed the induction of apoptosis after APC exposure. It is concluded that programmed cell death plays an important role in the cytotoxicity of APC against certain human leukemic cell lines. The antineoplastic profiles of APC with long alkyl chains render them attractive for further therapeutic application.
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  • 10
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The microstructures of single filaments of silver-sheathed (Bi,Pb)2Sr2Ca2Cu3O10+δ-multifilament tapes are investigated by polarized light and scanning electron microscopy. Large differences in the microstructure of filaments depending on their position in the cross section of the tape are found. The influence of this microstructure on the critical current density is investigated by magneto-optics. A drastic reduction of the critical current density by a factor of about 10 of the outer, compared to the filaments in the center of the tape is observed. © 1996 American Institute of Physics.
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