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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 55 (1977), S. 1165-1171 
    ISSN: 1432-1440
    Keywords: Haemophilia A ; Haemorrhagic diathesis ; Thrombocytopathy ; Hämophilie A ; Hämorrhagische Diathese ; Plättchenfunktionsstörung
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über 2 Patienten mit Hämophilie A berichtet, bei denen sich während einer operationsbedingten hochdosierten Substitutionstherapie mit antihämophilem Globulin vorübergehend eine Thrombozytenfunktionsstörung mit manifester hämorrhagischer Diathese einstellte. Zum Zeitpunkt der Blutung waren bei ausreichend hoher Faktor VIII-Aktivität der Fibrinogenwert im Plasma auf 1700 mg-% und der Gehalt an Faktor VIII-assoziiertem Antigen auf über 600% der Norm angestiegen. Da es während der Substitution zu keiner Zeit gelang, Fibrinogenspaltprodukte oder Komponenten des antihämophilen Globulins mit üblichen Methoden nachzuweisen, wird — unterstützt durch „in vitro“-Untersuchungen der Thrombozytenaggregation — die Ansicht vertreten, daß die Beladung der Plättchenoberfläche mit überschüssig zugeführten Proteinen ursächlich mit der thrombozytären Funktionsstörung zusammenhängt. Die Bedeutung kleinmolekularer und dialysierbarer Faktor VIII-Komponenten wird ebenso wie die Möglichkeit immunpathologischer Vorgänge diskutiert.
    Notes: Summary This report describes two patients with haemophilia A who developed a transient thrombocytopathy with haemorrhagic diathesis during post-operative high-dose replacement therapy with antihaemophilic globulin. At the time of the bleeding the factor VIII-activity was in the normal range in both patients. The fibrinogen level, however, was elevated to 1700 mg-% and the factor VIII-associated antigen rose to more than 6-fold. At no time of replacement therapy with antihaemophilic globulin could either fibrinogen split products or fragments of the factor VIII-protein be detected by the usual methods. In view of the results of the thrombocyte aggregation experiments the authors postulate a disturbance of platelet function at the level of the membrane surface due to an overload of increased amounts of circulating proteins. Both the possible interference of dialysable factor VIII-components and the role of immunpathologic phenomena are discussed.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Archives of gynecology and obstetrics 183 (1953), S. 503-506 
    ISSN: 1432-0711
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. S50 
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukaemia ; Bone marrow transplantation ; Chemotherapy ; Risk factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty-five years ago over 90% of children with acute lymphoblastic leukaemia (ALL) died of this disease. Dramatic improvement has been achieved since then by employing risk-adapted, aggressive polychemotherapy protocols. More than 90% of children with ALL treated according to, for example BFM-protocols, have nowadays cure rates in the range of 70%–80%. However, 10% of patients do not initially respond adequately to standard induction chemotherapy. They are characterized by distinct chromosomal abnormalities such as translocation (9; 22) or combinations of early treatment failure and other risk factors as cytogenetic abnormalities, lineage-specific surface markers or tumour load at diagnosis. In this group of patients in first complete remission and certainly in the vast majority of relapsed patients, allogeneic bone marrow transplantation (BMT) has evolved as an alternative approach allowing further intensification of myeloablation and the introduction of an additional antileukaemic alloreactivity. Nevertheless, the decision for a marrow transplant in children has to be made very carefully because of a significant increase in treatment related mortality and BMT-specific risks like acute and chronic graft-versus-host disease with a critical iatrogenic chronic morbidity. This is even more evident, if mismatched or unrelated transplants are being considered. The indications for one or the other treatment modality according to the current BFM strategy are discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 301-303 
    ISSN: 1432-1076
    Keywords: Congenital ring constriction ; Aberrant tissue bands ; Amniotic band syndrome ; Disruption complex ; Autosomal-dominant multiple benign deep ring-shaped skin creases ; Neuroblastoma ; Cleft palate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two families with autosomal-dominant transmission of benign ring-shaped skin creases: a father and his son with cleft palate and localized neuroblastoma in one family and a father and his two daughters-one with median cleft palate-in the second family, who showed circular skin creases on both arms and fingers, which almost disappeared in later life. Multiple benign deep ring-shaped skin alterations in a newborn of a third family are suggestive for a new dominant mutation. Our new observations should not be confused with amniotic ring constrictions.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 127 (1978), S. 219-226 
    ISSN: 1432-1076
    Keywords: Hemihypertrophy ; Wilms' tumor ; Adrenocortical neoplasia ; Wiedemann-Beckwith syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A girl with hemihypertrophy and hamartomas, now 14 years old, had Wilms' tumor and subsequently developed adrenocortical carcinoma. The occurrence of the two tumors with the signs of an inborn defect of growth control supports the hypothesis that both tumors can be caused by the same etiologic factors, which are also teratogenic. An alternative explanation of induction of the second tumor by previous radio- and chemotherapy is discussed. Possible relationships between our case and the syndrome of Wiedemann and Beckwith are pointed out.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukemia ; Cognitive functions ; Psychomotor speed ; School achievements
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Several studies have reported a decline in intelligence and cognitive functions in survivors of childhood acute lymphoblastic leukemia (ALL). Other investigators, however, have found no intellectual impairment in these children. Fifty-one long-term survivors of ALL, having been treated according to the protocols of the BFM Study Group from 1970 to 1979, were assessed retrospectively using neurophysical methods. The results were compared with those obtained from 30 patients with other malignancies, who had received neither radiation therapy to the central nervous system (CRT) nor any methotrexate during chemotherapy. Additionally, neurological examinations and cranial computed tomography (CCT) were performed. Neuropsychological examinations included verbal functions, intelligence (performance), psychomotor speed, motor skills and sensory integration. The results of verbal tests and the IQs, tested by nonspeed-related measures, were within normal limits in both groups. About one-third of all patients showed mild disturbances of psychomotor speed and motor skills. Children with leukemia had lower scores than those with solid tumors for nearly all tasks, but only tests for sensory integration revealed significant differences between former ALL patients and tumor patients. Furthermore, the following results were obtained related to different therapeutic modalities: (1) The higher total radiation doses had been during CRT (maximum 24 GY), the more neuropsychological functions were impaired, particularly motor accuracy and sensory integration. (2) These disturbances improved with the length of survival. Widening of subarachnoidal space was found in 33% of the CCT obtained. There was no correlation between the intellectual functions of the survivors and the CCT abnormalities. Neurological findings mainly consisted of slight fine motor disturbances. The results of the study suggest that psychomotor functions are more likely to be affected by antineoplastic therapy than are higher cognitive functions.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Mutation Research/Environmental Mutagenesis and Related Subjects 21 (1973), S. 179 
    ISSN: 0165-1161
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Mutation Research/Environmental Mutagenesis and Related Subjects 97 (1982), S. 437-447 
    ISSN: 0165-1161
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Cancer Genetics and Cytogenetics 38 (1989), S. 167 
    ISSN: 0165-4608
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 0165-4608
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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