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  • 1
    ISSN: 1569-8041
    Keywords: AT ; ataxia teleangiectasia ; Nijmegen-Breakage Syndrome (NBS) ; non-Hodgkin's lymphoma (NHL)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:Lymphoma and leukemia are the commonest malignantdiseases in patients with chromosomal breakage syndromes and immunodeficiency(Ataxia teleangiectasia (AT) and Nijmegen breakage syndrome (NBS)). Withimproved management of infections, malignant disease is more frequentlydiagnosed and has become one of the commonest causes of death in pediatric ATand NBS. Patients and methods:In three consecutive multicenter therapytrials for pediatric non-Hodgkin's lymphoma (NHL) (NHL-BFM), 1569 patientswith newly diagnosed NHL have been registered between 1986 and 1997. Ninepatients with AT (n = 5) and NBS (n = 4) were identified andanalysed. Results:Median age of patients with AT and NBS at diagnosis ofNHL was nine years. NHL-entities differed from non-AT/NBS-patients: diffuselarge B-cell lymphomas, n = 7 (78%); ALCL, n = 1;lymphoblastic T-cell lymphoma, n = 1. Cervical nodes, paranasalsinuses and epipharynx were the sites most frequently involved. Stages were:I and II in three patients, III in five and IV in one patient. All patientsreceived polychemotherapy according to tumor-entity and stage, none receivedradiation. Dose reductions according to individual tolerance concerned mainlyethotrexate, alkylating agents and epipodophyllotoxines. One patient died oftoxic complications, two patients relapsed and died, one patient suffered fromsecond malignancy. Five of nine patients are in 1. CCR after a medianfolluow-up of five years. Conclusions:Patients with AT and NBS suffer from rare entitiesof pediatric NHL. Curative treatment is possible and should be attempted.Intensity of therapy should be adjusted to individual risk factors andtolerance. Alkylating agents, epipodophyllotoxines should be omitted, dose ofMTX should be limited to 1 g/m2. Further cooperative trials usingstandardized approaches are required.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 116-121 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Onkologie ; Akute lymphoblastische Leukämie ; Skelettbeteiligung ; Skelettbeschwerden ; Key words Pediatric oncology ; Acute lymphoblastic leukemia ; Skeletal involvement ; Skeletal pain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Introduction: Children with ALL frequently present with skeletal pain at the time of diagnosis. We retrospectively analyzed the incidence and clinical features of skeletal pain and the correlation between pain and radiographic findings. Patients: Eighty children with ALL aged ≥3 years (45 m, 35 f) who were diagnosed and treated at the University Children’s Hospital Würzburg between 1974 and 1995 were included in the study. All patients received a radiographic skeletal survey at diagnosis. Results: Only 19 of 46 children with skeletal lesions complained about skeletal pain. Eleven of 30 children presenting with skeletal pain showed no radiographic skeletal lesions. The highest rate of skeletal pain was found in patients with areal osteolytic lesions (80%). Radiographic lesions of the lower extremities were associated with skeletal pain in 78% of all cases. Patients with skeletal pain presented with lower peripheral blast counts, with a smaller spleen size and more frequently with common-ALL (90%). 17% of ALL patients with skeletal pain and severe radiographic lesions had only minor alterations in blood count and normal size of abdominal organs. Frequently, this pain was correctly interpreted only after a longer (p〈0.05) history, indicating diagnostic difficulties in these patients. Conclusions: A long history of skeletal pain has to be considered as a leading symptom especially in ALL patients lacking typical hematological features. We recommend X-ray examination especially of the lower extremities and a bone marrow analysis in all patients with persisting skeletal pain of unknown origin.
    Notes: Zusammenfassung Fragestellung: Kinder mit akuter lymphatischer Leukämie (ALL) geben bei der Diagnosestellung häufig Skelettbeschwerden an. Wir untersuchten retrospektiv die Inzidenz und klinische Ausprägung von Skelettbeschwerden sowie die Korrelation zwischen Beschwerdebild und radiologischen Skelettläsionen bei ALL-Diagnose. Methode: Der röntgenologische Skelettstatus von 80 ALL-Patienten eines Alters ≥3,0 Jahre (45 m, 35 w), die zwischen 1974 und 1995 an der Universitäts-Kinderklinik Würzburg behandelt wurden, wurde von einem Kinderradiologen reevaluiert. Ergebnisse: Nur 19 von 46 Patienten mit radiologischen Skelettläsionen klagten über Skelettbeschwerden. 11 von 30 Patienten mit Skelettbeschwerden boten keine radiologischen Auffälligkeiten. Die meisten Skelettbeschwerden fanden sich bei Patienten mit flächigen Osteolysen (80%). Skelettbeschwerden im Bereich der unteren Extremitäten waren am häufigsten (78%) von radiologischen Skelettläsionen begleitet. 17% der Patienten boten Skelettbeschwerden als Leitsymptom ihrer ALL ohne Blutbildveränderungen oder Hepatosplenomegalie. Trotz ausgeprägter radiologischer Skelettveränderungen erfolgte die richtige Deutung der Skelettbeschwerden spät, gemessen an einer längeren Anamnesedauer (p〈0,05) der Patienten. Die für das Kindesalter typische aleukämische Verlaufsform der ALL mit niedriger peripherer Blastenzahl, geringer Splenomegalie und Common-Typ war mit Skelettbeschwerden und radiologischen Skelettläsionen assoziiert. Schlußfolgerungen: Länger bestehende Skelettbeschwerden sind ein wichtiges Leitsymptom der kindlichen ALL, insbesondere dann, wenn typische hämatologische Befunde noch nicht oder nur diskret ausgeprägt sind. Neben einer Röntgenuntersuchung des Skeletts, besonders der unteren Extremität, sollte zum Ausschluß einer ALL bei Persistenz unklarer Skelettbeschwerden auch eine Knochenmarkpunktion erfolgen.
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  • 3
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: In den letzten Jahrzehnten hat sich der Stellenwert der Strahlentherapie bei der Behandlung pädiatrischer Tumoren gewandelt. Fortschritte auf dem Gebiet strahlentherapeutischer Techniken und Kenntnisse über notwendige Dosierungen für eine maximale Tumorkontrolle bei minimaler Nebenwirkungsrate (Dosis-Wirkungs-Beziehungen) erlauben eine effiziente und schonende Bestrahlung mit Anhebung der Überlebenszeiten bei gleichzeitiger Reduktion von Therapiefolgen, ohne jedoch die Metastasierungstendenz wesentlich zu beeinflussen. Die Einführung von Chemotherapien erreichte eine zusätzliche systemische Tumorkontrolle, sodass heute bei der überwiegenden Mehrheit der Erkrankungen Heilungsraten erzielt werden, die 70% übersteigen (Tabelle 1). Während im Erwachsenenalter vorwiegend Tumoren epithelialen Ursprungs im Vordergrund stehen, werden Tumoren im Kindesalter vorwiegend durch Neoplasien embryonaler Genese gebildet. Diese zeigen eine erhöhte Empfindlichkeit gegenüber ionisierender Strahlung und chemotherapeutischen Substanzen. Hierdurch werden die im Vergleich zum Erwachsenenalter deutlich erhöhten Heilungsraten erklärt. Gleichzeitig reagiert aber der wachsende Organismus sensibler auf zytotoxische Agenzien. Einige chemotherapeutische Substanzen können zwar durch einen strahlensensibilisierenden Effekt die Tumorkontrolle verbessern, erhöhen jedoch gleichzeitig das Risiko für akute, maximale Nebenwirkungen und Spätfolgen (Tabelle 2). Hierdurch können nicht nur einzelne Organfunktionen beeinträchtigt werden, sondern auch die gesamte Entwicklung des betroffenen Kindes. Die Dosisschwelle für die Toleranz des kindlichen Gewebes v. a. der sich entwickelnden Organe liegt häufig unter den Dosierungen, die für die lokale Tumorkontrolle notwendig sind, und ändern sich durch eine zusätzliche Chemotherapie. Daher werden spezielle Anforderungen an die Strahlentherapie gestellt, um Normalgewebe und v. a. Risikoorgane zu schonen.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 665-668 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Sinusoidale Hämangiomatose ; Milz ; Kasabach-Merritt-Syndrom ; Key words Sinusoidal hemangiomatosis ; Spleen ; Kasabach-Merritt syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a 13-month-old girl who developed Kasabach-Merritt syndrome because of sinusoidal hemangiomatosis of the spleen. The combination of consumption coagulopathy and tumor of the spleen suggested a vascular process. Discussion: For a tumor of the spleen, sinusoidal hemangiomatosis has to be considered as differential diagnosis. Splenectomy is the treatment of choice when consumption coagulopathy occurs.
    Notes: Zusammenfassung Berichtet wird über eine 13 Monate alte Patientin, bei der sich aufgrund einer sinusoidalen Hämangiomatose ein Kasabach-Merritt-Syndrom entwickelte. Die Kombination von Gerinnungsstörung und Milztumor legte bereits den Verdacht auf einen vaskulären Prozeß nahe. Diskussion: Obwohl äußerst selten, muß die sinusoidale Hämangiomatose im Kindesalter in die Differentialdiagnose der Milztumoren mit eingeschlossen werden. Im Fall einer assoziierten Gerinnungsstörung ist die Splenektomie die Therapie der Wahl.
    Type of Medium: Electronic Resource
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  • 5
    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
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Der Onkologe 4 (1998), S. 608-617 
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Die Astrozytome hohen Malignitätsgrades (Grad III, IV) sind mit etwa 30% die häufigsten und zugleich aggressivsten Hirntumoren gliösen Ursprungs und führen unbehandelt innerhalb weniger Wochen zum Tode. Neben der primär einzusetzenden Operation ist der Stellenwert der postoperativen perkutanen Strahlentherapie in der Behandlung der malignen Gliome unbestritten. Derzeit gibt es keinen gesicherten Hinweis, daß eine Chemotherapie Operation und Strahlentherapie ersetzen kann, so daß die malignen Gliome wegen ihres aggressiven Wachstums und der damit verbundenen schlechten Prognose unverändert eine Herausforderung an den Radioonkologen stellen.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    World journal of urology 10 (1992), S. 100-106 
    ISSN: 1433-8726
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Between June 1980 and June 1988, 373 patients with Wilms' tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). In all, 11% of the patients were diagnosed as a result of routine checkup examinations. The tumor volume was 〈400 ml in 47.3% of cases and 〉400 ml in 52.7%. Overall, 78.7% of the patients had a standard-type Wilms' tumor, 7% had a low-grade malignancy lesion and 14.3% had a high-grade-malignancy tumor. Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. In all, 32% of the children received preoperative treatment and 68% underwent primary surgery. Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms' tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method). Radiotherapy was performed in 113 of the 218 protocol patients. Children demonstrating a clear-cell histology subtype did not show the poor prognosis attributed to this histology in the literature. Of the children with low-grade variants, 19 of 24 are free of disease. About 70% of all children with Wilms' tumor in the Federal Republic of Germany were entered in this study (47 of probably 68 new cases recorded annually nationwide×8 years). Of the 47 patients evaluated per year, 38 survived disease-free, whereas 9 per year either suffered from a disease or perished. The overall results are very satisfying. Additional treatment modifications can be achieved only through further studies. Not only for this reason is it recommended that Wilms' tumors be treated at large institutions. Wilms' tumor is the most common solid childhood tumor; 6.5% of all malignancies occurring in children are nephroblastomas. About 70 new cases are diagnosed every year in the Federal Republic of Germany. The therapy of Wilms' tumors depends on various factors such as the clinical stage and histologic subtype of the tumor and the age of the patient. New information for optimization of the therapy can only be gathered by means of a nationwide study. We report on the clinical data and treatment results of 373 children. The aim of this study was to elucidate the relationship between the prognosis and the clinical stage and histologic subtype of the tumor as well as the age of the patients. In addition, the importance of pre- and/or postoperative radiotherapy was studied. Furthermore, the dose and duration of treatment with chemotherapeutic agents was evaluated.
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  • 8
    ISSN: 1432-0584
    Keywords: Keywords PBSC mobilization failure ; Poor mobilizers ; Bone marrow harvest
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We assessed autologous bone marrow (BM) harvest and hematologic recovery after high-dose chemotherapy (HDCT) in patients who failed to achieve peripheral blood stem cell (PBSC) mobilization. One hundred and ninety-three patients with germ cell tumor, malignant lymphoma, sarcoma or medulloblastoma were scheduled for HDCT. In 123 patients, PBSC were mobilized by disease-specific chemotherapy plus granulocyte colony-stimulating factor (G-CSF). In 110/123 patients (89%) with circulating CD34+ cell counts ≥10/μl, sufficient hematopoietic autografts were collected (group A). In 13/123 patients (11%) with peripheral CD34+ cell counts 〈10/μl, PBSC harvesting was not performed (group B). These latter patients were classified as "poor mobilizers" and underwent second-line BM harvest at a median of 46 (range 10–99) days after mobilization failure. Seventy patients with first-line BM harvest (group C) acted as historical controls. Ten patients from group B proceeded to HDCT and nine were evaluable for hematopoietic reconstitution. Recovery to neutrophils 〉0.5×109/l was comparable with group C patients: 16 (range 9–34) days vs 13 (range 8–98) days. However, platelet (PLT) reconstitution 〉20×109/l was significantly slower, with a median of 35 (range 13–50) days as compared with 19 (range 9–148) days (P=0.0106) for control patients. Supportive care requirements, febrile days and length of hospital stay were not significantly different between the two groups of patients. We conclude that patients who fail to mobilize PBSC should be evaluated for second-line BM harvest. This approach may preserve the therapeutic option of HDCT for these patients.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Cancer chemotherapy and pharmacology 24 (1989), S. S40 
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The German Society of Pediatric Oncology (GPO) has studied the efficacy of high-dose ifosfamide with mesna uroprotection in patients with Ewing's sarcoma. A phase II trial of ifosfamide (IFO) (2 g/m2 per day, days 1–5) in eight patients with recurrent evaluable disease resulted in three partial and two complete responses lasting from 3 to 12 months (median, 6 months). In a second phase II trial in 15 patients, the combination of IFO and cisplatin (20 mg/m2 per day, days 1–5) resulted in 7 partial and 2 complete responses lasting from 3 to 32 months (median, 6 months). Consequently, in 1985 IFO was incorporated into first-line chemotherapy for newly diagnosed patients (replacing cyclophosphamide) and given in combination with vincristine, actinomycin D, and Adriamycin (VAIA) in patients considered to be at high risk for relapse. IFO was given at a dose of 3 g/m per day on days 1 and 2 as a 48-h continuous infusion, in combination with actinomycin D (0.5 mg/m2 per day on days 1–3) or Adriamycin (30 mg/m2 per day on days 1 and 2). The study was piloted from March to December 1985 and has been open since January 1986; 37 patients were entered during the pilot phase and 65 have been entered in the ongoing main trial since January 1986. At present, Kaplan-Meier disease-free survival projects that disease-free survival in patients with large primary tumors has improved compared with that reported for the previous CESS 81 trial. The toxicity of the VAIA regimen was comparable with that of the conventional vincristine, actinomycin D, cyclophosphamide, and Adriamycin (VACA) regimen used in the previous CESS 81 trial.
    Type of Medium: Electronic Resource
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