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  • 1
    Electronic Resource
    Electronic Resource
    Mechanisms of resistance to methotrexate in childhood acute lymphoblastic leukemia: circumvention of thymidylate synthase inhibition (1999)
    Springer
    Journal of cancer research and clinical oncology 125 (1999), S. 513-519 
    Details
    ISSN: 1432-1335
    Keywords: Key words Acute lymphoblastic leukemia ; Methotrexate polyglutamates ; Thymidylate synthase ; Salvage pathway ; Relapse
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Purpose: In about 25% of patients suffering from acute lymphoblastic leukemia (ALL) treatment failures occur that are most likely due to development of resistance to methotrexate (MTX). Blasts from patients with ALL were evaluated for MTX uptake, formation of long-chain MTX polyglutamates (MTX-Glu5+6), cytotoxicity and thymidylate synthase inhibition by MTX and compared to blasts from patients with acute myelogenous leukemia (AML). Methods: Radioactively labeled MTX-Glu n were analyzed by means of HPLC. Thymidylate synthase activity was measured by a tritium-release assay. Cytotoxicity was determined by trypan blue exclusion. Results: In most ALL blasts (n = 9) large amounts of MTX-Glu5+6 (1.06–7.03 pmol/107cells) and high cytotoxicity (43.5%–92.7%) were found, while in others small amounts of MTX-Glu5+6 (0.0–0.39 pmol/107cells) caused only weak cytotoxicity (6.0%–27.9%) (n = 5, 2 relapsed patients). Resistance to MTX in blasts from AML patients (n = 5) was also caused by reduced synthesis of MTX-Glu5+6 (0.0–0.42 pmol/107cells). In contrast, some ALL blasts (n = 7, 4 relapsed patients) were able to survive MTX treatment despite large amounts of MTX-Glu5+6 (1.5–5.05 pmol/107cells) and extensive thymidylate synthase inhibition. Conclusions: Since the majority of ALL patients were examined at first diagnosis, an inherent mechanism of resistance seems most likely. We propose a mechanism based on the switch of thymidylate synthesis to the salvage pathway.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004320050310
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  • 2
    Electronic Resource
    Electronic Resource
    Bare lymphocyte syndrome — combined immunodeficiency and neutrophil dysfunction (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 700-704 
    Details
    ISSN: 1432-1076
    Keywords: Bare lymphocyte syndrome ; HLA expression ; Severe combined immunodeficiency syndrome ; Neutrophil dysfunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal β-2-microglobulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, −DQ, −DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959526
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  • 3
    Electronic Resource
    Electronic Resource
    The BFM-protocol for HIV-negative Burkitt's lymphomas and L3 ALL in adult patients: a high chance for cure (1992)
    Springer
    Annals of hematology 65 (1992), S. 201-205 
    Details
    ISSN: 1432-0584
    Keywords: Burkitt's lymphoma ; Acute lymphoblastic leukemia, B-type ; Tumor lysis syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary During a period of 9 years we used the pediatric BFM-NHL protocol for treatment of 14 adult patients with Burkitt's lymphoma or L3 acute lymphoblastic leukemia. Ten of 14 patients obtained a complete remission including 5/8 with stage-IV disease or B-ALL. After a median follow-up of 55 months none of these ten patients relapsed. The projected survival after 8 years is 71%. Toxicity was moderate, with one early death; a tumor lysis syndrome occurred in four patients. From our experience we conclude that the BFM-NHL protocol is very effective in adult patients, with a high cure rate and acceptable toxicity, even in advanced stages of disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01703945
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  • 4
    Electronic Resource
    Electronic Resource
    Long-term results of the co-operative German–Austrian–Swiss osteosarcoma study group's protocol COSS-86 of intensive multidrug chemotherapy and surgery for osteosarcoma of the limbs (1998)
    Springer
    Annals of oncology 9 (1998), S. 893-899 
    Details
    ISSN: 1569-8041
    Keywords: chemotherapy ; cisplatin ; ifosfamide ; intraarterial therapy ; osteosarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: In an effort to intensify osteosarcoma therapy, systemic ifosfamide was added pre- and postoperatively to an already aggressive three-drug regimen. In a subgroup of patients, loco-regional treatment intensification was attempted by using the intraarterial route to give cisplatin. Patients and methods: Patients ≤40 years at diagnosis of a localised, de novo high-grade central extremity osteosarcoma were eligible for inclusion into study COSS-86 if registered within three weeks from biopsy. Doxorubicin, high-dose methotrexate, and cisplatin were given to all patients. Patients who fulfilled one or more of three defined high-risk criteria received early systemic treatment intensification by adding ifosfamide as the fourth agent. Preoperatively, these high-risk patients received cisplatin either intraarterially or intravenously. Results: 171 eligible patients were entered, of which 128 were stratified into the high-risk group. When all 171 were analysed by intention-to-treat, actuarial overall and event-free survival rates at ten years were 72% and 66%, respectively. No benefit of intraarterial cisplatin application was detected. Cumulative treatment toxicity was considerable. Conclusions: In a multicenter setting, intensive treatment of osteosarcoma according to protocol COSS-86 led to long-term disease-free survival for two thirds of patients. We saw no benefit of using the intraarterial route to administer cisplatin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1008391103132
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  • 5
    Electronic Resource
    Electronic Resource
    Durchführung eines neuen Therapiekonzepts für Nephroblastome im Bereich der Gesellschaft für Pädiatrische Onkologie und Hämatologie SIOP 9/GPOH (1997)
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 128-135 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Nephroblastom ; Klinische Studie ; Präoperative Chemotherapie ; Diagnostische Sicherheit ; Therapiereduktion ; Key words Nephroblastoma ; Clinical trial ; Pre-operative chemotherapy ; Diagnostic accuracy ; Reduction of therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In a new study the intensity of treatment used in the former Wilms' Tumor Study of the „Gesellschaft für Pädiatrische Onkologie und Hämatologie“ (GPOH) since 1980 had to be reduced maintaining the good results. Methods: 1989 the strategy of the International Society of Pediatric Oncology (SIOP) with pre-operative chemotherapy after diagnostic imaging was adopted. The pretreatment should reduce the tumorsize to allow complete tumor resection in most patients. Results: Of 505 patients enrolled in the study over 5.25 years from 78 participating centers, 486 had a nephroblastoma, 14 another malignant tumor and 5 a benign renal lesion. Of 438 patients with nephroblastoma aged between 0.5 and 16 years 85.4 % received pre-operative chemotherapy. Compared to the prior study the percentage of patients with irradiation (22.8 %) was lowered and the cumulative dose of Adriamycin for higher stages and unfavourable histology reduced. The 91 % probability of crude survival after 3 years for all nephroblastoma patients was similiar to the former study. Conclusions: The concept of general pre-operative chemotherapy for nephroblastoma was accepted by GPOH with evident reduction of treatment intensity compared to the prior study.
    Notes: Zusammenfassung Hintergrund: Die intensive Therapie der von der Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) ab 1980 durchgeführten Wilmstumorstudie sollte in der Folgestudie unter Wahrung der guten Ergebnisse reduziert werden. Methode: 1989 wurde das Therapiekonzept der International Society of Pediatric Oncology (SIOP) mit präoperativer Chemotherapie nach bildgebender Diagnose übernommen, wobei der Tumor durch die Vorbehandlung verkleinert wird, damit er bei einem Großteil der Kinder vollständig entfernbar ist. Ergebnisse: Bei 505 in 5,25 Jahren aus 78 Kliniken gemeldeten Patienten hatten 486 ein Nephroblastom, 14 ein anderes Malignom und 5 eine benigne renale Läsion. Von 438 gemeldeten Nephroblastompatienten zwischen 0,5 und 16 Jahren erhielten 85,4 % präoperativ eine Chemotherapie. Der Anteil bestrahlter Nephroblastompatienten (22,8 %) und die kumulative Adriamycindosis bei hohen Stadien bzw. hoher Malignität wurden im Vergleich zur Vorstudie reduziert bei ähnlicher Überlebenswahrscheinlichkeit von 91 % nach 3 Jahren. Schlußfolgerung: Das Konzept einer generellen präoperativen Chemotherapie zur Nephroblastombehandlung ist in der GPOH mit gutem Ergebnis und deutlicher Therapiereduktion im Vergleich zur Vorstudie durchzuführen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050114
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  • 6
    Electronic Resource
    Electronic Resource
    Therapie von Infektionen in der pädiatrischen Onkologie (1997)
    Springer
    Der Onkologe 3 (1997), S. S44 
    Details
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Bei Immunsuppression infolge zytostatischer Behandlung ist Fieber häufig das einzige Zeichen einer beginnenden schweren und mitunter lebensbedrohlichen Infektion. Empirisch wird daher wenige Stunden nach Fieberbeginn mit einer i. v.-antibiotischen Breitspektrumtherapie in Form einer Monotherapie oder Kombinationstherapie begonnen [12, 19]. Durch die Therapie mit einem β-Laktam-Antibiotikum allein oder in Kombination mit einem Glykopeptidantibiotikum oder einem Aminoglycosidantibiotikum konnte bei erwachsenen Patienten eine hohe Wirksamkeit in der Behandlung der febrilen Neutropenie belegt werden [8, 10, 18]. Umfangreiche klinische Studien zur Wirksamkeit gleichartiger Therapieschemata in der pädiatrischen Onkologie sind selten. In einer Multizenterstudie wurde geprüft, ob eine Kombinationstherapie mit Ceftazidim und Teicoplanin als Initialtherapie einer antibiotischen Sequentialtherapie bei Kindern und Jugendlichen mit febriler Neutropenie effektiv ist.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00008333
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  • 7
    Electronic Resource
    Electronic Resource
    Morbus Coats bei einem Jungen mit DiGeorge-Syndrom (1997)
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 1061-1065 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter DiGeorge-Syndrom ; Morbus Coats ; Leukokorie ; Retinoblastom ; Amaurose im Kindesalter ; Key words DiGeorge-Syndrom ; Coats’ disease ; Leukocoria ; Retinoblastoma ; Amaurosis in childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Coats’ disease is characterized by teleangiectatic and aneurysmal changes of the retinal vessels with secondary intra- and subretinal exudates. In most cases vision can be preserved by therapy at an early stage. In rare, advanced cases a total retinal detachment causes amaurosis. This case is the first description of Coats’ disease associated with DiGeorge-Syndrome. We report the case of an eight year old boy, who suffered from headache after ear surgery (tympanoplastic type I). Retinoblastoma could be exluded and Coats’ disease was diagnosed by ophthalmoscopy, computertomography and magnetic resonance imaging. Discussion: Until today the cause of Coats’ disease is unknown. The coincidence with DiGeorge-Syndrome seems to be accidental.
    Notes: Zusammenfassung Der Morbus Coats ist charakterisiert durch teleangiektatische und aneurysmatische Gefäßveränderungen der Retina und sekundäre intra- und subretinale Exsudate. Meist ist durch frühzeitige Therapie der Visus zu erhalten. In seltenen, fulminant verlaufenden Fällen können eine vollständige Amotio retinae und Erblindung auftreten. Dieser Fall ist die erste Beschreibung eines Morbus Coats bei einem DiGeorge-Syndrom. Wir berichten über einen 8jährigen Jungen mit einem DiGeorge-Syndrom, bei dem nach einer Tympanoplastik Typ I starke Kopfschmerzen auftraten. Durch Ophthalmoskopie, Computertomographie und Kernspintomographie konnten ein Retinoblastom ausgeschlossen und ein Morbus Coats diagnostiziert werden. Diskussion: Die Ursache des Morbus Coats ist bis heute nicht geklärt. Das Zusammentreffen mit dem DiGeorge-Syndrom ist wahrscheinlich zufällig.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050204
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  • 8
    Electronic Resource
    Electronic Resource
    Arachnoidalzyste als Ursache eines Hypernatriämie-Hypodypsie-Syndroms (1998)
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 603-607 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Hypernatriämie ; Hypernatriämie-Hypodypsie-Syndrom ; Arachnoidalzyste ; Stereotaxie ; Kinder ; Key words Hypernatremia ; Hypodipsic-hypernatremia-syndrome ; Arachnoid cyst ; Stereotactic surgery ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In rare cases a hypernatremia is caused by cerebral dysregulation. Case: We describe a 3,5 year old girl who had a hypodipsic-hypernatremia-syndrome (serum sodium concentration 194 mmol/l) caused by an arachnoid cyst in front of the pons. Therapy: In a stereotactic puncture a catheter was implanted. Aspiration of the cyst liquid did reduce the size of the cyst. For two years now no other treatment is given. Hypernatremia did not occur again. Discussion: To our knowledge this is the first case of a hypodipsic-hypernatremia-syndrome caused by an arachnoid cyst, which was cured by a single operation.
    Notes: Zusammenfassung Hintergrund: In seltenen Fällen werden Hypernatriämien durch zerebrale Regulationsstörungen verursacht. Fall: Wir berichten von einem 3,5 Jahre alten Mädchen, bei dem ein Hypernatriämie-Hypodypsie-Syndrom (Serumnatrium 194 mmol/l) durch eine präpontine Arachnoidalzyste verursacht wurde. Therapie: Durch eine stereotaktische Operation wurde ein Katheter implantiert. Der Zysteninhalt wurde aspiriert und die Zyste dadurch verkleinert. Seit 2 Jahren trat ohne medikamentöse Therapie keine Hypernatriämie mehr auf. Diskussion: In diesem Fall konnte nach unserem Wissen erstmals ein Hypernatriämie-Hypodypsie- Syndrom, das durch eine Arachnoidalzyste verursacht wurde, durch eine Operation geheilt werden.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050300
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  • 9
    Electronic Resource
    Electronic Resource
    Tetra- und Pentacarbonyl-Derivate der Metalle Chrom, Molybdän und Wolfram mit sauerstoff- und schwefelverbrückten Distibanen sowie mit Chlorodiphenylstiban als KomplexligandenProfessor Otto J. Scherer zum 60. Geburtstage gewidmet (1993)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 619 (1993), S. 1991-1997 
    Details
    ISSN: 0044-2313
    Keywords: Pentacarbonyl(diorganostibine)chromium, -molybdenum, -tungsten complexes ; Tetracarbonyl(diorganostibine)chromium and -molybdenum complexes ; Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Tetra- and Pentacarbonyl Compounds of Chromium, Molybdenum and Tungsten with Oxygen- and Sulfurbridged Distibines and with Chlorodiphenylstibine as Complex LigandsThe penta- and tetracarbonyl complexes of chromium, molybdenum and tungsten with the stibines Ph2SbCl and (Ph2Sb)2X (X = O and S) are obtained by photochemical reaction of the hexacarbonyls in thf and by thermic ligand substitution of (η4-C7H8)M(CO)4 (M = Cr, Mo) with stibines.In the case of (Ph2Sb)2X monodentate or bidentate coordination is possible.
    Notes: Die Synthese von Penta- und Tetracarbonylen der Metalle der 6. Nebengruppe mit Antimonliganden des Typs Ph2SbCl und (Ph2Sb)2X (X = O und S) erfolgt durch photochemische Umsetzung der Metallhexacarbonyle in THF bzw. durch thermische Ligandsubstitution aus (η4-C7H8)M(CO)4 (M = Cr, Mo) mit den Antimonliganden.Bei Verwendung der Chelatstibane (Ph2Sb)2X läßt sich sowohl eine monodentate als auch eine bidentate Koordination verwirklichen.
    Additional Material: 2 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19936191206
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  • 10
    Electronic Resource
    Electronic Resource
    Eisen- und Mangankomplexe mit sauerstoff-, schwefel- und methylen-verbrückten Distibanen und mit Chlorodiphenylstiban als Liganden (1993)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 619 (1993), S. 2061-2065 
    Details
    ISSN: 0044-2313
    Keywords: Dicarbonylbis(triphenylphosphite)(stibine)iron complexes ; Bis[dicarbonylbis(triphenylphosphite)iron](stibine) complexes ; Dicarbonyl(η5-methylcyclopentadienyl)(stibine)manganese complexes ; Bis[dicarbonyl(η5-methylcyclopentadienyl)manganese](stibine) complexes ; Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Complexes of Iron and Manganese with Oxygen-, Sulfur-, and Methylene-bridged Distibines and with Chlorodiphenylstibine as LigandsThe photochemical reaction of CO3Fe[P(OPh)3]2 and of MeCpMn(CO)3 with the stibines Ph2SbCl and (Ph2Sb)2X (X = O, S and CH2) yields mononuclear complexes (CO)2[P(OPh)3]2FeL and °Cp(CO)2MnL (L = Ph2SbCl, (Ph2Sb)2X) and the stibine bridged binuclear complexes °CO)2[P(OPh)3]2Fe{(Ph2Sb)2X}Fe(CO)2[P(OPh)3]2 and °Cp(CO)2Mn{(Ph2Sb)2X}Mn(CO)2MeCp.
    Notes: Durch photochemische Umsetzung von (CO)3Fe[P(OPh)3]2 und von MeCpMn(CO)3 mit Anti-monliganden des Typs Ph2SbCl und (Ph2Sb)2X (X = O, S und CH2) können Einkernverbindungen (CO)2[P(OPh)3]2FeL und MeCp(CO)2MnL (L = Ph2SbCl, (Ph2Sb)2X sowie die über Distibanliganden verbrückten Zweikernverbindungen (CO)2[P(OPh)3]2Fe{(Ph2Sb)2X}Fe(CO)2[P(OPh)3]2 und MeCp(CO)2Mn{(Ph2Sb)2X}Mn(CO)2MeCp synthetisiert werden.
    Additional Material: 4 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19936191215
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