[Results of treatment of osteosarcoma in children according to the SFOP-94 protocol in the experience of the Polish Paediatric Solid Tumour Study Group]. / Wyniki leczenia dzieci chorych na miesaka kosciopochodnego wedlug protokolu SFOP-94 w materiale Polskiej Grupy Pediatry Cznej ds. Leczenia Guzów Litych.

Between April 1994 and December 1999, 34 children aged from 5 to 20 years (23 females and 11 males) suffering from osteosarcoma, were treated according to the SFOP-94 protocol. The primary preoperative chemotherapy consists of adriamycin and high-dose methotrexate administration. There were 28 patients with non-metastatic tumours of the extremities and 6 children presented disseminated disease with pulmonary metastases. The primary localization included femur - 20 patients, tibia - 9 patients and humerus - 5 patients. In 26 patients limb-salvage surgery was applied. The programme of chemotherapy was changed in 4 children because of toxicity of methotrexate (1 patient) and progression of disease (3 patients). 26 out of 34 (76,5 %) children are alive including 24 out of 28 patients with localized disease. EFS calculated according to Kaplan-Meier analysis was 60 % at 67 months.

[G-CSF and GM-CSF in treatment of neutropaenia after chemotherapy in children with neoplasms]. / G-CSF i GM-CSF w leczeniu neutropenii po chemioterapii nowotworów u dzieci.

A total number of 608 cycles of G-CSF and/or GM-CSF was applied in 280 patients aged from 6 months to 20 years during neutropaenia associated with chemotherapy of children's neoplasms (NHL-124, NBL-42, RMS-36, Nephroblastoma-18, Osteosarcoma-17, Ewing's Sarcoma-14, Hepatoblastoma-6, Neurofibrosarcoma-6, PNET-5, Medulloblastoma-3, Fibrohistiocytoma-3, Angiosarcoma-2, other - 4). G-CSF - Neupogen (Filgastrim, Hoffman La Roche - 492 cycles) and GM-CSF - Leucomax (Molgramostim, Shering Plough - 116 cycles) were administered 5 mg/kg/day s.c. Forty one children with malignancies (NHL -21 cases, solid tumours -17) treated before cytokines were in use served as a control group. Our study demonstrated that G-CSF and GM-CSF therapy, gives a shorter period of neutropaenia, reduction of the number of febrile days, decreased frequency of infection and shortened its duration.

[The analysis of failures in therapy of osteosarcoma in children treated according to SFOP-94 protocol in the studies of Polish pediatric solid tumors treatment group]. / Analiza niepowodzen leczenia miesaka kosciopochodnego u dzieci wedlug protokolu SFOP-94 w materiale Polskiej Pediatrycznej Grupy ds. Leczenia Guzów Litych.

Between December 1989 and April 1998 twenty eight children aged from 5 to 20 years (18 female and 10 male) suffering from osteosarcoma were treated according to the OS-SFOP-94 protocol. Twenty four patients presented with localized tumor of extremities and four with pulmonary metastases. The majority of primary tumors exceeded 150 ml of volume. The primary preoperative chemotherapy consisted of adriamycin (70 mg/m2 every four weeks) and high-dose methotrexate (12 g/m2 every week). In 20 patients limb-salvage surgery was applied, in three children--amputation and in one child tibia resection with genu arthrodesis was applied. Five of 28 patients died, one because of treatment related infection, 2 non-responders with metastatic osteosarcoma due to progressive disease, and one because of local relapse with pulmonary metastasis non-responding to therapy, one because of treatment refusal. Twenty one from 25 children are alive from 5 to 51 months. Event frae survival of children with localized disease calculated according to Kaplan-Meier analysis was 64.17% in the 51st month. The main cause of failure in the treatment of osteosarcoma in children is primary and secondary progression of disease. The toleration and results of treatment for osteosarcoma in children according to the OS-SFOP-94 is satisfactory.

[The efficacy of G-CSF and GM-CSF in the adjunctive treatment of infections complicating chemotherapy of solid tumors in children. Report of Polish Pediatric Leukemia/Lymphoma Treatment Group]. / Skutecznosc G-CSF i GM-CSF w leczeniu wspomagajacym zakazen wiklajacych chemioterapie guzów litych u dzieci. Raport PPG/LBC.

Total number of 306 cycles of GM-CSF-Leucomax Sandoz (5 mg/kg/day s.c.) and/or G-CSF Filgrastim Hoffmann-La Roche (5-10 mg/kg/day s.c.) was applied in 146 children aged from 0.5-18 years during neutropenia associated with chemotherapy of solid tumours. Seventeen children with malignancies served as a historical control group. Our study have demonstrated after both G- and GM-CSF therapy shorter period of neutropenia, reduction of the number of febrile days and a decreased frequency of infectious complications and infection's duration.

[The effectiveness of G-CSF and GM-CSF in the adjunctive treatment of infections complicating chemotherapy of non-Hodgkin's lymphoma in children. Report of Polish Pediatric Leukemia/Lymphoma Treatment Group]. / Skutecznosc G-CSF i GM-CSF w leczeniu wspomagajacym zakazen wiklajacych chemioterapie nieziarniczych chloniaków zlosliwych u dzieci. Raport PPG/LBC.

Total number of 252 cycles of GM-CSF-Leucomax Sandoz (5 mg/kg/day s.c.) and/or G-CSF Filgrastim Hoffmann-La Roche (5-10 mg/kg/day s.c.) was applied in 124 children aged from 0.5-20 years during neutropenia associated with chemotherapy of non-Hodgkin's lymphoma (NHL). Twenty four children with NHL treated according to the same chemotherapy protocol but without G-CSF and GM-CSF served as a control group. Our study have demonstrated the good efficacy of both G-CSF and GM-CSF therapy. They shortened the period of neutropenia, reduced the number of febrile days, infection's duration and decreased the frequency of infectious complications.

[Significance of immunological system examinations in the clinical course of Langerhans cell histiocytosis in children]. / Ocena stanu ukladu odpornosciowego w przebiegu histiocytozy komórek Langerhansa u dzieci.

The Langerhans cell histiocytosis (LCH) is an enigmatic disease, usually occurring in the young. Its etiology is unknown, its pathogenesis is not correctly understood and the clinical course is unpredictable. In the years from 1975 to 1998 in Department of Paediatric Haematology and Oncology (Wroclaw University School of Medicine) 32 children with diagnosis of the Langerhans Cell Histiocytosis (LCH) were treated. The study group included 19 boys and 13 girls, aged from 3 weeks to 14 years, median age 7.5 years. Investigations of immunological system were performed in the examined group. In all children lymphocytes' subsets were examined. We examined apoptosis with ELISA test in 3 patients, in 3--marked antigen CD44, in 12 marked sVCAM-1 by ELISA method. IL-1 in growing cell supernatants was determined in 17 children. In 15 children lower IL-1 production was observed before the beginning of therapy in comparison with control group. In all patients decreased concentrations of lymphocytes T were noted, but in 8 children lower CD4/CD8 rate was observed. In the study group higher expression of CD44 was noted. In 3 patients cell apoptosis was normal. In 12 patients lower expression of sVCAM-1 was observed. Abnormal expression of adhesion molecules may influence the pathogenesis of LCH. Disorders of immunological system were observed in the patients with LCH.

[Coexistence of Langerhans cell histiocytosis and Niemann-Pick disease in children: a case report]. / Wspólistnienie histiocytozy komórek Langerhansa z choroba Niemanna-Picka--opis przypadku.

Langerhans cell histiocytosis (LCH) and Niemann-Pick disease are very rare diseases. Both LCH and Niemann-Pick disease are distinguished by complex etiology, pathogenesis and broad spectrum of clinical manifestations. In our study we presented case report of 2.5 year old girl in who the coexistence of both diseases was noted. General symptoms that led to diagnosis were: hepatosplenomegaly, cholestatic jaundice, cutaneous lesions and mental development retardation.