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  • 1
    ISSN: 1432-1076
    Keywords: Trisomy G-21 ; Phosphofructokinase ; Deficiency in Erythrocytes ; Gene Locus ; Glycerophosphatedehydrogenase ; Erythrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In den Erythrocyten eines Falles von Trisomie G-21 konnte im Gegensatz zu bisher vorliegenden Berichten eine Verminderung der Aktivität von Phosphofructokinase (PFK) gefunden werden. Daneben wurde eine hohe Aktivität der Glycerophosphatdehydrogenase und in geringerem Ausmaß auch anderer Enzyme nachgewiesen. Die mit dem PFK-Mangel in Zusammenhang stehenden Stoffwechselstörungen sowie die Frage der genetischen Lokalisation dieses Enzyms werden diskutiert.
    Notes: Abstract A deficiency of erythrocyte-phosphofructokinase has been demonstrated in a case of Trisomy-G-21. This is in contrast to findings of other authors who reported an increase of erythrocyte-PFK in cases of Down's syndrome. The metabolic changes caused by PFK deficiency and the problem of genetic localization of this enzyme are discussed. In addition an increased activity of glycerophosphate-dehydrogenase and, to a lesser degree, some other enzyme was found in the patients red blood cells.
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  • 2
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Erythrocyte metabolism studies in a case with hereditary spherocytosis only showed minimal deviations of the activity of the enzymes of glycolysis from the normal values. The formation of lactate was increased, the intracellular sodium concentration was increased and the intracellular potassium concentration was decreased. The methemoglobin level of the erythrocytes was increased. After splenectomy, which resulted in a good clinical and hematological remission, lactate formation and the abnormal electrolyte and methemoglobin level of the erythrocytes returned to normal.
    Notes: Zusammenfassung Untersuchungen des Stoffwechsels der Erythrozyten bei einem Fall von hereditärer Sphärozytose ergaben nur geringe Abweichungen der Aktivität der Fermente der Glykolyse von der Norm. Die Bildung von Laktat war gesteigert, der Gehalt an intrazellulärem Natrium vermehrt und an Kalium vermindert; der Methämoglobingehalt der Erythrozyten war vermehrt. Nach der Splenektomie, die zu einer guten klinischen und hämatologischen Remission führte, normalisierte sich die Laktatbildung und der abnorme Elektrolytund Methämoglobingehalt der Erythrozyten.
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  • 3
    ISSN: 1432-0584
    Keywords: Key words Mastocytosis ; Multiple myeloma ; Mast cells ; Tryptase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Mastocytosis is a term used for a spectrum of disorders characterized by abnormal growth and accumulation of mast cells. The cutaneous variants of the disease have to be distinguished from systemic mastocytosis (SM), in which at least one extracutaneous organ is involved. In contrast to cutaneous mastocytosis, SM is often associated with another hematologic neoplasm. In most cases clonal myeloid malignancies such as a myeloproliferative or myelodysplastic syndrome occur. In a few cases of SM, however, clonal lymphoid disorders have been described. We here report on a case of SM associated with multiple myeloma. At first presentation, the 48-year old female patient showed monoclonal IgGλ gammopathy and bone marrow (BM) mastocytosis, but no BM plasma cell infiltrates. Eight years later, the patient presented with BM mastocytosis and overt multiple myeloma. The co-existence of myeloma and mastocytosis was demonstrable by staining serial BM sections with antibodies against mast cell tryptase, CD68R, and the plasma cell marker VS38c. Interphase FISH analysis of BM sections revealed a numeric gain of chromosome 5 and chromosome 7 in the plasma cells but not in the mast cell infiltrates, thereby confirming the presence of two different neoplastic cell populations. To our knowledge, this is the first report describing the co-existence of multiple myeloma and mastocytosis.
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  • 4
    ISSN: 1432-0584
    Keywords: Chronic myeloid leukemia ; Chronic and acute phase of CML ; Chromosomal findings ; Clinical course
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In an attempt to relate karyotype evolution to clinical and hematological data serial chromosomal analyses were performed in 31 patients with chronic myeloid leukemia (CML), both in chronic and acute phases. Our results in Philadelphia chromosome (Ph1)-positive CML are in line with karyotype profiles described in the literature. In addition, we report on chromosomal findings in 4 cases of Ph1-negative disease, one presenting with an iso17q chromosome in the acute phase, an aberration otherwise typical for acute transformation in Ph1-positive CML. The same chromosomal abnormality was observed in a small population of Ph1-negative cells present in one of two patients with mixed Ph1-positive/Ph1-negative CML. The first case of a female patient with the loss of a sex chromosome in Ph1-positive cells is reported. Two patients with unusually long and mild chronic phases despite the presence of trisomy 8 in their karyotypes are described. Our findings suggest that the order of appearance of additional chromosomal changes of CML is of prognostic significance for the progression and the clinical picture of the disease.
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  • 5
    ISSN: 1432-0584
    Keywords: Key words Chronic B-lympohocytic leukemia ; Secondary MDS/AML ; Clonal analysis ; IgH gene rearrangement ; DXS 255/M27β
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The development of myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML) has rarely been observed in patients with chronic B-lymphocytic leukemia (B-CLL). So far, the discussion concerning the pathogenesis of the simultaneous occurrence of these two malignancies has been speculative, opposing the theory of two separate malignant clones to the theory of a common stem cell malignancy. We describe the case of a 77-year-old woman who developed MDS after 8 years of an indolent course of B-CLL. The diagnosis of MDS was based on bone marrow (BM) morphology, showing the typical picture of a refractory anemia with excess of blasts (RAEB). The clinical course of MDS was aggressive, terminating in AML within only 6 months. Immunophenotyping of BM and peripheral blood (PB) cells revealed a CD34+/CD13+/CD33–/CD19– blast cell population and a CD19+/CD5+ B-cell population with kappa light chain restriction. Molecular analysis of PB and BM demonstrated the presence of an immunoglobulin heavy chain (IgH) gene rearrangement by polymerase chain reaction (PCR) amplification of genomic DNA with three different pairs of consensus primers. Cell-sorting experiments showed that the IgH gene rearrangement was present only in the CD19+/CD34– B-cell population, but not in the CD34+/CD19– blast cells. Furthermore, X-chromosome inactivation pattern analysis revealed two differently methylated cell populations. These experiments demonstrate the concomitant existence of two different clones in a patient with CLL-MDS/AML.
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  • 6
    ISSN: 1432-0584
    Keywords: CHOP-Chemotherapy-first line treatment ; Kiel-classification ; unfavorable prognosis NHL ; prognostic factors ; risk factor score
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 58 NHL-patients (9 large cell centrocytic, 18 centroblastic, 16 immunoblastic, 15 lymphoblastic lymphomas) were treated immediately after diagnosis with CHOP-chemotherapy regardless of the extent of disease. Because of the advanced age of the majority of patients (median age 61 years, range 22–85 years) a reduced dose in the first two cycles was administered. Statistically significant prognostic variables influencing survival were the following: histologic subtypes according to the Kiel-classification (p〈0,05), B-symptoms (p〈0,001), blood sedimentation rate (p〈0,02) and LDH (p〈0,0005). With regard to prognosis there was no difference between patients over 60 years of age and younger ones (p〈0,4). Patients achieving complete remission survived significantly longer (p〈0,0001). Ann Arbor stages were of limited value, since patients with CS II disease and accumulation of risk factors (B-symptoms, abdominal disease, bulky tumor masses) showed a poorer outcome than patients with CS III who did not have these risk factors. A risk factor score summarizing features influencing prognosis is described and might be a useful tool in stratifying the heterogenous group of NHL with unfavorable prognosis.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Research in experimental medicine 162 (1974), S. 341-346 
    ISSN: 1433-8580
    Keywords: Fructose ; Purine synthesisde novo ; Adenine nucleotides ; Rat liver ; Rat muscles ; Hyperuricemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The intravenous infusion of fructose (0.7 and 2.4 g/kg/30 min) increases the incorporation of14C-glycine into rat liver adenine nucleotides significantly as compared to controls treated with 0.9% NaCl. A decrease of the total adenine nucleotide content of the livers occurs concomitantly. In contrast to its effect upon liver tissue fructose neither effects the content of adenine nucleotides nor the incorporation of glycine into these nucleotides in skeletal muscle of rats. — The results support the hypothesis that large quantities of fructose cause an increasedde novo synthesis of purine nucleotides in the liver. However, they do not as yet present clear cut evidence as to whether this is a direct effect of fructose or is secondary to the depletion of adenine nucleotides.
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  • 8
    ISSN: 1591-9528
    Keywords: Experimental nephrocalcinosis ; High-energy-phosphates ; Experimentelle Nierenverkalkung ; Energiereiche Phosphate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Durch 0,5 mg DHT/100 g KG wurde bei Ratten eine Nephrocalcinose erzeugt. 24, 48, 72 und 96 Std nach der DHT-Zufuhr wurden die Nieren elektronenoptisch untersucht und das ATP, ADP und AMP unter Anwendung der In situ-Gefrierstoppmethode in den Organen bestimmt. Trotz höhergradiger morphologischer Veränderungen in den Zellen traten keine signifikanten Unterschiede im ATP-, ADP- und AMP-Gehalt in den Nieren auf. Im Frühstadium der DHT-induzierten Nephrocalcinose werden die energiereichen Phosphate ATP, ADP und AMP in der Gesamtniere durch den Verkalkungsvorgang nicht beeinflu\t.
    Notes: Summary The authors investigated the concentration of high-energy-phosphates ATP, ADP and AMP in rat kidneys during experimentally induced nephrocalcinosis (0.5 mg DHT/100 g body weight) and compared the results with fine structural alterations of the tubulus. Despite extensive intracellular calcification on PAS positive masses in cytoplasma vacuoles and calcification of mitochondria no significant difference in ATP, ADP and AMP concentration from untreated control animals could be observed. In the early stages of experimentally induced calcification of the kidney no marked metabolic disturbances occur prior to the development of cell necrosis.
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  • 9
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    Journal of Mathematical Physics 29 (1988), S. 1486-1497 
    ISSN: 1089-7658
    Source: AIP Digital Archive
    Topics: Mathematics , Physics
    Notes: Corrections and clarifications are made of some past treatments of the variational nature of the eigenfrequency calculation for dispersion equations and new results are presented. The main conclusions are the following: (1) Any relation between a normal mode and its dual must be consistent with the fact that the boundary conditions satisfied by the normal mode may differ from the adjoint boundary conditions satisfied by the dual. This will affect whether or not a given bilinear form will yield a variational result for the eigenfrequency. (2) If a dispersion matrix is constructed from the dispersion operator by using left and right basis functions that satisfy homogeneous boundary conditions on the dual eigenfunction and the eigenfunction, respectively, then generally a second-order accurate eigenfrequency is obtained by solving the matrix form of the dispersion equation. (3) When solving for the normal modes in terms of perturbation potentials, the adjoint boundary conditions are gauge dependent. For cases where the adjoint boundary conditions allow only the trivial solution for the dual eigenfunction, it may be possible to obtain variational results for the eigenfrequency by requiring that the trial functions for the normal mode and its dual satisfy variational boundary conditions.
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  • 10
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Berichtet wird über Adeninnukleotiduntersuchungen (ATP, ADP, AMP) in normaler menschlicher Skeletmuskulatur (M. quadr. fem.). Biopsietechnik, Gewebeaufarbeitung und biochemische Bestimmungsmethode werden beschrieben.
    Notes: Summary The results of quantitations of ATP, ADP, and AMP in normal human skeletal muscle (M. quadratus femoris) are presented. The techniques of muscle biopsy, tissue preparation and the biochemical methods are discussed.
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