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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 992-999 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Kongenitale dyserythropoetische Anämie ; Ineffektive Erythropoese ; Erythrozytenmembran ; Eisenüberladung ; Key words Congenital dyserythropoietic anemia ; Ineffective erythropoiesis ; Iron overload ; Red cell membrane
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The congenital dyserythropoietic anemias represent a group of rare inborn errors with ineffective erythropoiesis as the predominant mechanism of anemia. They are primarily classified into three types on the basis of characteristic abnormalities of the erythroblasts as shown by light and electron microscopy. There are distinct associated features such as pattern of heredity, cell kinetics and abnormalities of the cell membrane. In addition, patients with apparent CDA of heterogenous phenotype are observed, that cannot be attributed to any of the three types. Recently variant genes have been localized to different regions on chromosome 15 or 22. Cases of CDA were reported from many ethnics, in particular in families of meditarranean origin. Many patients load iron necessitating iron depletion. Gallstones are a frequent complication and are observed in childhood or adolescence. Therapeutic measures such as splenectomy, treatment by Interferon α or allogeneic bome marrow transplantation depend on the type and the severity of clinical expression, which shows large variations both within and between affected kindreds.
    Notes: Zusammenfassung Unter dem Begriff der kongenitalen dyserythropoetischen Anämien (CDA) wird eine geno- und phänotypisch heterogene Gruppe seltener angeborener Erkrankungen der Erythropoese zusammengefaßt. Sie sind durch eine Ineffektivität der Erythropoese und charakteristische morphologische Veränderungen der Erythroblasten gekennzeichnet, die eine Einteilung in 3 Typen erlauben. Damit assoziierte Merkmale betreffen den Erbgang, die Zellkinetik, Veränderungen der Erythrozytenmembran und die unterschiedliche Lokalisation des varianten Gens. Die Pathogenese ist nur teilweise bekannt. Bei dem meisten Patienten kommt es, teilweise bereits im Kindesalter, zur Gallensteinbildung und zur sekundären Hämochromatose, die eine Eisenentzugsbehandlung erfordert. Therapeutische Maßnahmen wie Splenektomie, Interferon-α-Behandlung oder allogene Knochenmarktransplantation richten sich nach dem CDA-Typ und der sehr unterschiedlichen Schwere der Erkrankung.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Der Onkologe 5 (1999), S. 637-641 
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Die onkologischen Erkrankungen gehören wegen ihrer Häufigkeit und ihrer Bedeutung für Morbidität und Mortalität zu den wesentlichen Herausforderungen, denen sich Absolventen des Medizinstudiums heute und in der Zukunft zu stellen haben. Die altersstandardisierte krebsspezifische Mortalität liegt in Deutschland bei 280/100.000 Einwohner jährlich; dies entspricht 250.000 Krebstodesfällen pro Jahr. Da etwa jede zweite Krebserkrankung geheilt wird, ist die Zahl der jährlich neudiagnostizierten Fälle etwa doppelt so hoch. Aufgrund des steilen Anstiegs des Krebsrisikos nach dem 60. Lebensjahr ist, trotz aller Bemühungen um eine verbesserte Primärprävention, mit weiter steigender Lebenserwartung eine weitere Zunahme wahrscheinlich. Globale Angaben zur Prävalenz sind nicht verfügbar und sagen wegen der unterschiedlichen natürlichen Prognose verschiedener Krebsformen wenig aus. Zweifellos ist unabhängig von der Inzidenzentwicklung jedoch auch hier mit einem weiteren Anstieg zu rechnen, da die Zahl der für viele Jahre erfolgreich palliativ behandelbaren, aber nicht geheilten Krebspatienten zunimmt. Die Onkologie ist weltweit ein Schwerpunktthema und bedarf der. Gestaltung eines onkologischen Curriculums in der ärztlichen Ausbildung.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0584
    Keywords: Autologous transfusion ; Platelets ; Immunization ; Acute leukemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Seventy-eight transfusions of autologous platelets were given to eight alloimmunized patients receiving curative chemotherapy for acute leukemia. Platelets were collected at regeneration of hematopoiesis after a chemotherapy cycle, cryopreserved with 5% dimethylsulfoxide in liquid nitrogen, and retransfused during bone marrow aplasia following the next treatment cycle. The in vitro platelet recovery after freezing, thawing, and washing was 85 ±4%. The in vivo corrected count increment 1 h after autologous platelet transfusions was 11±5×109/l. With the exception of moderate urticaria and slight nausea each after one transfusion, no immediate or chronic side effects occurred. The bleeding time was shortened and hemorrhage during bone marrow aplasia was prevented in all alloimmunized patients by autologous platelet transfusions.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Key words De novo AML ; Adults ; HD-Ara-C/DNR consolidation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A total of 149 consecutive de novo AML patients aged 50 years or less (median age = 37 years) were enrolled in this prospective multicenter trial initiated in May 1985. All patients received the same induction and early consolidation therapy with daunorubicin (DNR), cytosine arabinoside (Ara-C), and etoposide (DAV). High-dose Ara-C/DNR therapy included Ara-C at 3 g/m2, in 12 doses (HD-Ara-C/DNR I) and eight doses (HD-Ara-C/DNR II), followed by DNR 30 mg/m2 for 3 days. A complete remission (CR) was achieved in 104 (70%) patients; 61 complete responders received at least one cycle with HD-Ara-C/DNR. If those patients who were transplanted in first CR (n = 26), were not considered, the median relapse-free-survival (MRFS) of the remaining 78 patients was 15 months, with a probability of relapse-free survival (RFS) at 116 months of 30% (95% CI, 20–40%) after a median follow-up of 95 months. The MRFS of the HD-Ara-C/DNR consolidated patients was 25 months, with a probability of RFS at 116 months of 37% (95% CI, 24–50%). If all patients who were transplanted (n = 44) were not considered, the median survival time (MST) was 18 months with a probability of being alive at 118 months of 24% (95% CI, 16–33%). MST of the HD-Ara-C/DNR consolidated patients was 58 months with a survival probability of 46% (95% CI, 31–60%) at 118 months. Prognostic factor analysis did not reveal any significant influence of age, sex, FAB subtype, white blood cell count, hemoglobin level, thrombocyte count, LDH, or response to the first induction course on RFS of the HD-Ara-C/DNR consolidated patients. In summary, HD-Ara-C/DNR consolidation can improve the long-term outcome of a subgroup of de novo AML patients. Further improvement of the outcome seems to depend on the identification of patients with an inferior outcome under that strategy who might benefit from alternative treatment strategies.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0584
    Keywords: Key words Autologous transfusion ; Platelets ; Immunization ; Acute leukemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Seventy-eight transfusions of autologous platelets were given to eight alloimmunized patients receiving curative chemotherapy for acute leukemia. Platelets were collected at regeneration of hematopoiesis after a chemotherapy cycle, cryopreserved with 5% dimethylsulfoxide in liquid nitrogen, and retransfused during bone marrow aplasia following the next treatment cycle. The in vitro platelet recovery after freezing, thawing, and washing was 85±4%. The in vivo corrected count increment 1 h after autologous platelet transfusions was 11±5×109/l. With the exception of moderate urticaria and slight nausea each after one transfusion, no immediate or chronic side effects occurred. The bleeding time was shortened and hemorrhage during bone marrow aplasia was prevented in all alloimmunized patients by autologous platelet transfusions.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0584
    Keywords: De novo AML ; Adults ; HD-Ara-C/DNR consolidation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A total of 149 consecutive de novo AML patients aged 50 years or less (median age = 37 years) were enrolled in this prospective multicenter trial initiated in May 1985. All patients received the same induction and early consolidation therapy with daunorubicin (DNR), cytosine arabinoside (Ara-C), and etoposide (DAV). High-dose Ara-C/DNR therapy included Ara-C at 3 g/m2, in 12 doses (HD-Ara-C/DNR I) and eight doses (HD-Ara-C/DNR II), followed by DNR 30 mg/m2 for 3 days. A complete remission (CR) was achieved in 104 (70%) patients; 61 complete responders received at least one cycle with HD-Ara-C/DNR. If those patients who were transplanted in first CR (n=26), were not considered, the median relapsefree-survival (MRFS) of the remaining 78 patients was 15 months, with a probability of relapse-free survival (RFS) at 116 months of 30% (95% CI, 20–40%) after a median follow-up of 95 months. The MRFS of the HD-Ara-C/DNR consolidated patients was 25 months, with a probability of RFS at 116 months of 37% (95% CI, 24–50%). If all patients who were transplanted (n=44) were not considered, the median survival time (MST) was 18 months with a probability of being alive at 118 months of 24% (95% CI, 16–33%). MST of the HD-Ara-C/DNR consolidated patients was 58 months with a survival probability of 46% (95% CI, 31–60%) at 118 months. Prognostic factor analysis did not reveal any significant influence of age, sex, FAB subtype, white blood cell count, hemoglobin level, thrombocyte count, LDH, or response to the first induction course on RFS of the HD-Ara-C/DNR consolidated patients. In summary, HD-Ara-C/DNR consolidation can improve the long-term outcome of a subgroup of de novo AML patients. Further improvement of the outcome seems to depend on the identification of patients with an inferior outcome under that strategy who might benefit from alternative treatment strategies.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 71 (1995), S. 205-205 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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