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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. 432-434 
    ISSN: 1432-1076
    Keywords: Ribs ; Leukaemia ; Hodgkin disease ; Neuroblastoma ; Wilms tumour
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P〈0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukaemia ; Childhood ; Combination chemotherapy ; Rotation of drugs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A frequent change of drug combinations may circumvent drug resistance in the treatment of patients with acute lymphoblastic leukaemia (ALL). In study COALL 85/89 201 children with high-risk ALL were randomized to receive over a period of 8 months rotational chemotherapy with six drug combinations given either in slow rotation (SR) (each combination given twice in succession) or in rapid rotation (RR) (cach combination given once with a repetition of the drug combinations). Treatment of central nervous system leukaemia consisted of cranial irradiation and intrathecal methotrexate. Both SR and RR treatment groups were then given continuation chemotherapy of oral 6-mercaptopurine and methotrexate until 2 years after the date of diagnosis. The 9-year eventfree survival (EFS) rate for the whole group is 69%±3%, and the survival rate 75%±3% at a median follow up of 5.8 years. Failure to achieve remission at day 28 was the most important prognostic factor (EFS 12%±7% vs. 75%±3% in the remission group). In the RR group, 11/100 patients were not in remission at day 28 opposed to 7/101 patients in the SR group. Children aged 〈1 year (6/6 relapses) or aged 〉=10 years had a worse prognosis (EFS 64%±5% vs. 77%±4% in patients 1–10 years old). After 5 years EFS was inferior in the RR group attributable to a significantly higher relapse rate in children with a WBC〉=100/nl. The EFS at 9 years for all patients, however, is similar in both groups (SR 72%±5% vs. RR 67±5%).
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Key words Acute lymphoblastic ; leukaemia ; Childhood ; Combination chemotherapy ; Rotation of drugs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A frequent change of drug combinations may circumvent drug resistance in the treatment of patients with acute lymphoblastic leukaemia (ALL). In study COALL 85/89 201 children with high-risk ALL were randomized to receive over a period of 8 months rotational chemotherapy with six drug combinations given either in slow rotation (SR) (each combination given twice in succession) or in rapid rotation (RR) (each combination given once with a repetition of the drug combinations). Treatment of central nervous system leukaemia consisted of cranial irradiation and intrathecal methotrexate. Both SR and RR treatment groups were then given continuation chemotherapy of oral 6-mercaptopurine and methotrexate until 2 years after the date of diagnosis. The 9-year event-free survival (EFS) rate for the whole group is 69% ± 3%, and the survival rate 75% ± 3% at a median follow up of 5.8 years. Failure to achieve remission at day 28 was the most important prognostic factor (EFS 12% ± 7% vs. 75% ± 3% in the remission group). In the RR group, 11/100 patients were not in remission at day 28 opposed to 7/101 patients in the SR group. Children aged 〈 1 year (6/6 relapses) or aged ^ 10 years had a worse prognosis (EFS 64% ± 5% vs. 77% ± 4% in patients 1–10 years old). After 5 years EFS was inferior in the RR group attributable to a significantly higher relapse rate in children with a WBC ^ 100/nl. The EFS at 9 years for all patients, however, is similar in both groups (SR 72% ± 5% vs. RR 67 ± 5%). Conclusion The COALL 85/89 treatment protocol with early intensive therapy and rotation of different drug combinations offers long-term disease-free survival for children with high-risk ALL. A continuous 4-week exposure to one drug combination may be necessary to achieve optimal results, especially in children with a high leukaemic cell burden.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Beckwith-Wiedemann syndrome ; Congenital hemihypertrophy ; Medullary sponge kidney ; Wilms tumour
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The occurrence of a Wilms tumour in a 4-year-old girl with bilateral medullary sponge kidney, Beckwith-Wiedemann syndrome and congenital hemihypertrophy demonstrates the close relationship between these disorders. Another six cases from the literature with congenital hemihypertrophy and with medullary sponge kidney are discussed, two of them also developed intra-abdominal neoplasm.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 2 (1986), S. 121-125 
    ISSN: 1433-0350
    Keywords: Brain tumors ; Children ; Hydrocephalus ; Hygroma ; Porencephaly
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty-seven children and adolescents with brain tumors were examined by magnetic resonance imaging (MRI) after tumor resection. The typical changes and complications after surgery and chemotherapy, as well as the corresponding MRI findings, are discussed. Typical examples of boundary-layer lesions, tumor recurrences, hydrocephalus, porencephalic cysts, and hygromas are given.
    Type of Medium: Electronic Resource
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