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  • 1
    Electronic Resource
    Electronic Resource
    Coagulation factors and proteinase inhibitors in the plasma of children with acute lymphoblastic leukoses (1984)
    Springer
    Journal of molecular medicine 62 (1984), S. 1165-1169 
    Details
    ISSN: 1432-1440
    Keywords: Acute lymphoblastic leukaemia in children ; Therapy ; Coagulation factors ; Proteinase inhibitors ; Fibronectin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The thrombocyte count, the factor XIII (F XIII) activity, the concentration of fibrinogen (F I), prothrombin (F II), fibronectin (CIG), albumin and the proteinase inhibitors antithrombin III (AT III), alpha 2-macroglobulin (A2M), alpha 1-antitrypsin (A1A) and Cl-esterase inactivator (C $$\bar 1$$ -INA) were determined in ten children with acute lymphoblastic leukaemia (ALL). Changes due to the disease and to therapy were observed. Before the start of treatment the patients had thrombocytopenia secondary to the disease, and the proteinase inhibitors — especially C $$\bar 1$$ -INA and A1A — were raised. During the induction phase the thrombocyte count rose but there was also a marked increase in the concentration of F II and CIG. During the consolidation phase there was a general fall in protein concentration underl-asparaginase medication. The cause was attributed to a disorder of protein synthesis. The concentration of the factors studied rose again during maintenance therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01712183
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  • 2
    Electronic Resource
    Electronic Resource
    Wilms tumours diagnosed by routine physical examinations (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 174-174 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445937
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  • 3
    Electronic Resource
    Electronic Resource
    Association of rib anomalies and malignancy in childhood (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 432-434 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01959357
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  • 4
    Electronic Resource
    Electronic Resource
    Randomized comparison of rotational chemotherapy in high-risk acute lymphoblastic leukaemia of childhood — follow up after 9 years (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 640-648 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01957144
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  • 5
    Electronic Resource
    Electronic Resource
    Randomized comparison of rotational chemotherapy in high-risk acute lymphoblastic leukaemia of childhood – follow up after 9 years (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 640-648 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01957144
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  • 6
    Electronic Resource
    Electronic Resource
    Physical performance in long-term survivors of acute leukaemia in childhood (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 464-467 
    Details
    ISSN: 1432-1076
    Keywords: Key words Childhood cancer ; Leukaemia ; Late effects ; Anthracyclines Exercise testing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The aim of this study was to assess the physical performance in long-term survivors of acute leukaemia in childhood and to evaluate the effects of anthracycline therapy. Electrocardiography, echocardiography and spiroergometry were carried out on 56 patients aged 9–28 years, of whom 44 patients had been treated with 15–483 mg/m2 doxorubicin (or equivalent). Acute leukaemia had been diagnosed 1.5–16 years earlier. Of the patients 75% reached normal maximal oxygen uptake, 69% normal oxygen uptake at the anaerobic threshold and 95% normal maximal work rate. Of the patients 75% achieved adequate values for maximal heart rate and 78% normal blood lactate concentration. No difference was seen between patients treated with and without anthracyclines. Conclusion The results of this study provide little evidence for cardiopulmonary impairment in long-term survivors of ALL. Both the cardiac function, as evaluated by ECG and echocardiography, and the physical performance in spiroergometry are normal in a large number of these patients. Anthracycline treatment does not appear to have a negative effect on these parameters.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050854
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  • 7
    Electronic Resource
    Electronic Resource
    Immunological investigations in two brothers with ataxia telangiectasia Louis-Bar (1976)
    Springer
    European journal of pediatrics 122 (1976), S. 93-102 
    Details
    ISSN: 1432-1076
    Keywords: Ataxia telangiectasia ; Autoimmune hemolytic anemia ; Immune deficiency disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Zwei von drei Brüdern (13 und 16 Jahre alt) zeigten das klinische Vollbild der Ataxia teleangiectatica Louis-Bar. Der ältere der Knaben leidet zusätzlich an einer autoimmun-hämolytischen Anämie, die im Zusammenhang mit anderen Immunmangel-syndromen, nicht aber bei Ataxia teleangiectatica bislang bekannt ist. Eingehende immunologische Untersuchungen bei den Brüdern zeigten eine Einschränkung von Zahl und Funktion der T-Zellen. Die Zahl der B-Lymphocyten war normal, darunter fanden sich auch IgA-positive Zellen, obwohl Serum-IgA fehlte. Es erscheint möglich, daß dieses Phänomen durch eine Reifungsstörung mit fehlender Differenzierung in IgA-synthetisierende Plasmazellen zustande kommt.
    Notes: Abstract Two of three brothers with the classical signs of ataxia telangiectasia were investigated for their immunological disorders at the ages of 13 and 16 years, respectively. The elder brother also suffers from autoimmune hemolytic anemia, a complication which has not yet been described in the course of ataxia telangiectasia. Immunological investigations made in both brothers showed a reduction in the number and function of T lymphocytes. The number of B lymphocytes was normal, among which there were cells staining for IgA, although serum IgA was absent. It seems possible that this phenomenon is caused by a disturbance in the process of maturation of lymphoid cells with a lack of differentiation into IgA-synthesizing plasma cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00466267
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  • 8
    Electronic Resource
    Electronic Resource
    Dermatoglyphische Untersuchungen bei Kindern mit embryonalen Tumoren (1975)
    Springer
    European journal of pediatrics 120 (1975), S. 101-110 
    Details
    ISSN: 1432-1076
    Keywords: Embryonic tumors ; Papillary ridges
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 60 Kinder mit embryonalen Tumoren wurden auf Veränderungen des Hautleistensystems hin untersucht. Es stellten sich verschiedene Unterschiede gegenüber der Normalpopulation heraus. Dies unterstreicht die embryonale Genese der verschiedenen Tumoren und stützt den Begriff des Mißbildungstumors. Embryonale Tumoren müssen als klinisch wichtigste Manifestation eines umfassenderen, in seinem ganzen Umfang aber sicherlich noch nicht vollständig erfaßten Fehlbildungssyndroms verstanden werden.
    Notes: Abstract Dermatoglyphics were analyzed in 60 children with embryonic tumors. In comparison with normal children, several differences were found. Thus, the embryonal origin of the different tumors is underlined and the hypothesis of embryonic tumors as malformations is sustained. These tumors seem to be the clinically most important manifestation of a much more comprehensive malformation syndrome, which is not yet known in all its details.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445159
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  • 9
    Electronic Resource
    Electronic Resource
    Fetal rhabdomyomatous nephroblastoma (1980)
    Springer
    European journal of pediatrics 133 (1980), S. 167-172 
    Details
    ISSN: 1432-1076
    Keywords: Nephroblastoma ; Wilms' tumor ; Nodular renal blastema ; Rhabdomyosarcoma ; Fetal rhabdomyoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fetal rhabdomyomatous nephroblastoma is a very rare variant of Wilms' tumor. The special clinical and histologic features of this variant are presented on the basis of a case seen in a boy who was 13 months old at nephrectomy 8 months ago and has remained healthy since then. The tumor chiefly consists of fetal striated muscle; it occurs predominantly in infancy and early childhood and is often bilateral. Angiography shows a relative paucity of vessels. The volume of the tumor (determined by ultrasonography) decreases only slightly after preoperative radio- and chemotherapy because of a predominance of mesenchymal structures. The finding of nodular renal blastema in renal parenchyma with an apparently normal gross appearance is an argument in favor of classifying this type of tumor in the group of nephroblastomas and may explain the tendency of fetal rhabdomyomatous nephroblastoma to occur bilaterally.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441587
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  • 10
    Electronic Resource
    Electronic Resource
    Editorial comment: Mass screening for early detection of neuroblastoma? (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 312-312 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442703
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