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Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.

OBJECTIVE: The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy. METHODS: Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age. Local tumour control was ensured by surgery and risk-adapted radiotherapy. RESULTS: From 1995 to 2002, 89 patients were registered in Austria. The 3-year event-free survival (EFS) and overall survival rates (OS) were 63% ± 6% and 71% ± 6%, respectively. 59/89 patients had localised RMS-like (rhabdomayosarcoma) STS (EFS 73% ± 7%), 14 had localised Non-RMS STS (EFS, 54% ± 16%) and 15 patients had metastatic disease at diagnosis (EFS, 33% ± 12%), 1 patient had fibromatosis. The EFS rates at 3 years in patients with localised RMS-like tumours according to risk group were 92% ± 8% for low and standard risk (12 patients) and 67% ± 8% for high risk (47 patients). Favourable primary tumour sites of nonmetastatic RMS-like STS i.e. orbit, head/neck nonparameningeal or genitourinary non-bladder/prostate were diagnosed in 15 patients (1 of 15 patients died). In 44 patients with unfavourable localisation such as parameningeal, genitourinary bladder/prostate, extremity and others, 7 deceased. The 3-year EFS according to histology in patients with RMS-like STS was 61% ± 11% for RME (embryonal RMS ) (28 patients) and 71% ± 15% for RMA (alveolar RMS) (10 patients). The most common treatment failure was local relapse occurring in 21% of patients in the high-risk group. CONCLUSION: Risk-adapted individualisation of treatment led to a reduction of chemotherapy in the low- and standard-risk group without compromising survival. The outcome of RME and RMA was similar in this cohort of patients. These preliminary results after a median observation time of 2.5 years confirm the CWS 96 strategy.

Objective To examine the usefulness of determining extended serum cytokine profiles in patients with juvenile rheumatoid arthritis (JRA), for the purpose of improving differential diagnosis and monitoring disease activity. Methods In a 2-year prospective study, serum levels of interleukin-1 beta (IL-1 beta), soluble IL-2 receptor (sIL-2R), IL-6, IL-8, tumor necrosis factor alpha (TNF alpha), and the p55 soluble TNF receptor (sTNFR) were repeatedly determined by enzyme-linked immunosorbent assay in 40 patients with JRA, 13 patients with postinfectious arthropathies, and 30 healthy controls. The data were compared with conventional parameters of inflammation, such as C-reactive protein (CRP), iron and hemoglobin levels, erythrocyte sedimentation rate (ESR), white blood cell (WBC) counts, and platelet counts. WBC subsets were analyzed by flow cytofluorometry. Results At the first visit and at the peak of inflammatory activity according to CRP levels and/or ESR, serum levels of sIL-2R, IL-6, and sTNFR in JRA patients correlated significantly with conventional inflammation indicators, whereas IL-1 beta, IL-8, and TNF alpha did not. No changes in leukocyte subset distribution were noted. Among the different clinical subtypes of JRA, sIL-2R, IL-6, and sTNFR values at the time of the initial visit showed a pattern similar to CRP, whereby patients with systemic disease exhibited by far the highest values. TNF alpha and IL-1 beta were variably elevated in certain JRA subtypes. Patients with postinfectious arthropathies showed elevated levels of CRP, sIL-2R, TNF alpha, and sTNFR, which did not differ significantly from levels in the various JRA subtypes with the exception of systemic disease. Detailed analysis of types I and II pauciarticular JRA revealed that levels of CRP, IL-1 beta, and TNF alpha were elevated in patients with type I disease. While these parameters were invariably normal in patients with type II disease, sTNFR and sIL-2R were still found to be significantly elevated. Followup studies suggested that persistently high sTNFR values are a better indicator of JRA activity than are measurements of other cytokines or CRP. Conclusion JRA is associated with significant and consistent changes in serum levels of inflammatory cytokines and soluble receptors. For the clinical monitoring of JRA, determination of levels of sTNFR, and to some extent sIL-2R, may be particularly useful, since these determinations yield information about subtype and/or activity of disease that is not available from conventional parameters of inflammation.

Polymorphonuclear leukocyte (PMN) migration is measured in whole blood in a migration chamber consisting of a membrane filter (3-microns pores, 140 microns thick) with an integrated chemoattractant depot (FMLP in solid form) attached to a plastic container. Control chambers lack FMLP (blanks). One test unit requires 300 microliters blood. Numbers and distribution of the PMN immigrants into the filters are determined microscopically. Altogether 26 measurements of PMN migration in five juvenile rheumatoid arthritis (JRA) patients with varying disease activity were compared with the reactions of a healthy control group (N = 32). Correlations were calculated with conventional laboratory parameters (WBC, PLT, BSR, CRP, Hgb, serum Fe) and disease activity. In comparison with healthy controls, PMNs of JRA patients generally show a markedly increased penetration depth into the filters irrespective the presence of the chemoattractant or the disease activity. Increased migratory reactions to FMLP in comparison to blanks were found during high disease activity only. The PMN penetration depth correlates positively with the CRP, and reciprocally with the Hgb blood levels. The migration assay combines fast and simple processing with good preservation of the genuine PMN activation state.

Three treatment techniques using two beam qualities have been compared on the basis of dose to the lens in prophylactic cranial irradiation. The dose to the lens and the globe was measured with thermoluminescent crystals in an anthropomorphic phantom and calculated by a computer-assisted planning system. A comparison was made of large field and small field techniques using 60Co and 8 MV photons. Modifications to the basic techniques studied included angulation of the gantry, angulation of the couch, and placement of an additional eye block close to the surface. The dose to the lens could be reduced to four percent of the midplane dose by applying the small-field technique combined with the use of 8 MV energy photons, by placing an additional block close to the surface, and by five degree occipitally angling the gantry, as well as rotating the treatment couch to account for the divergence of the beam. The use of 60Co produced an underdosage of the posterior segment of the globe in angled treatment techniques.

The purpose of this study was to examine patterns of peripheral T-cell-activation antigen expression after polyclonal in vitro stimulation in early stages of lymphoproliferative diseases. With 18 patients afflicted with recently diagnosed, non-leukemic stages of B and T cell lymphoma cytofluorimetric analysis was performed with peripheral blood lymphocytes (PBL) after 72 h in culture with and without phytohemagglutinin, using antibodies against the differentiation antigens CD3, CD8, CD4, CD16, CD19, CDw14, and the activation antigens interleukin-2 receptor (IL-2R, CD25), HLA-DR (DR), CD56 and transferrin receptor (TR). Compared to healthy controls and patients with other diseases, a very significant reduction of large T cells bearing activation markers was found in all lymphoma cases. Furthermore, a pronounced inhibition in the expression of the activation markers IL-2R and TR, but not of DR, was detected on CD3+ cells in phytohemagglutinin-stimulated PBL of all lymphoma cells independently of DNA synthesis, as measured by [3H]thymidine uptake. Determination of the natural-killer-cell-(NK)-associated antigens CD16 and CD56, available for our studies in a CD16 + CD56 combination kit, revealed, after phytohemagglutinin stimulation, significantly increased expression values in 8 lymphoma patients so far investigated, as compared to 12 healthy controls. Thus, polyclonal activation combined with cytofluorimetric screening of activation antigens seems to give useful information on the functional defect(s) of PBL in an early state of lymphoma, and may therefore be of considerable diagnostic value. The observed pattern of T cell activation antigen expression after phytohemagglutinin stimulation may give further clues to the understanding of immune dysfunction(s) associated with lymphoma.

41 Kinder mit intraventrikularer Hamorrhagie (IVH) wurden untersucht und die computertomographischen Ausgangsbefunde entsprechend der Gradierung von Papile in 4 Schweregrade gegliedert. Der Verlauf wurde computertomographisch und klinisch kontrolliert. Mit zunehmendem Schweregrad nahmen sowohl die Letalitatsrate als auch das Ausmas der computertomographischen Folgeveranderungen und der Defektheilungen (psychomotorische Entwicklungsverzogerung und psychoneurologisches Defektsyndrom) zu. Anhand von 3 Fallen wird gezeigt, das der computertomographische Befund lediglich einen prognostischen Teilaspekt darstellt. The investigation comprises 41 children with intraventricular hemorrhage (IVH). The CT-findings were divided into 4 groups, according to Papile. A CT and clinical follow-up study was performed on the survivors. The grade of IVH correlated with the rate of mortality and with neurological deficits. It is demonstrated in 3 cases that the CT-scan is only part of a prognostic aspect which is remarkable influenced by clinical events.

The distal tip of a catheter was accidentally severed in a 13-year-old girl when a polyethylene catheter was inserted into the right subclavian vein.

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.

Malignant Histiocytosis (MH) is a non hereditary rapidly fatal disease characterized by a neoplastic proliferation of atypical histiocytes, predominantly within lymphoid and haematopoeitic organs.Median duration of survival is two years after Adriamycin-based chemotherapy (CHOP) with complete remission rates from 57 % to 92 % (1). Despite early responsiveness of MH to Chemotherapy the disease is still frequently relapsing at the end or shortly after therapy. On the contrary Phillips et al.(2) report a disease free survival rate of 56+ months in a patient with MH who has been treated with cyc-lophosphamide, total body irradiation (TBI) and autologous bone marrow transplantation (ABMT).

Three children of two unrelated families developed signs of megaloblastic anaemia during their second year of life. They were shown to suffer from congenital vitamin B12-malabsorption. In contrast to most known children with the Imerslund Grasbeck-syndrome our patients had no proteinuria or aminoaciduria.