Heterogeneous spectrum of mutations in the Fanconi anaemia group A gene.

Fanconi anaemia (FA) is a genetically heterogeneous autosomal recessive disorder associated with chromosomal fragility, bone-marrow failure, congenital abnormalities and cancer. The gene for complementation group A (FAA), which accounts for 60-65% of all cases, has been cloned, and is composed of an open reading frame of 4.3 kb, which is distributed among 43 exons.

Systemizing childhood cancer care in Hungary: twenty-five years of progress.

Steady and significant progress has been made in the organization of Hungarian pediatric oncology. This has been done through the mobilization of social, scientific, medical, and economic resources of Hungary, a small nation. The lessons learned, and the measures adopted, may prove useful to other countries facing similar limitations and problems.

Effect of etoposide on the pharmacokinetics of methotrexate in vivo.

The effect of etoposide on the pharmakokinetics of methotrexate (MTX) was examined in vivo. High-dose (5g/m2/24 h) MTX therapy was combined with two etoposide (100mg/m2/ 1 h) infusions as a part of the medulloblastoma protocol developed in our department. Vepesid therapy was administered in two different schedules.

Recombinant human erythropoietin in the prevention of chemotherapy-induced anaemia in children with malignant solid tumours.

This prospective, randomised pilot study was designed to evaluate safety, feasibility and efficacy of recombinant human erythropoietin (rhEPO) in the prevention and treatment of chemotherapy-induced anaemia in children with solid tumours. 20 children (age 4-18 years) undergoing cyclic combination chemotherapy were randomised either to a control group or to receive rhEPO at a dose of 150 U/kg/dose subcutaneously three times/week for a minimum of 12 weeks or three chemotherapy cycles. Of 15 evaluable patients, 8 were randomised to the rhEPO group and 7 to the control group.

Comparative analysis of 8-oxo-2' -deoxyguanosine in DNA by 32P- and 33P-postlabeling and electrochemical detection.

8-Oxo-2'-deoxyguanosine (8-oxo-dG) is emerging as a useful marker for oxidative DNA damage. Reported basal levels determined by 32P-postlabeling (PPL) method were 10-fold or more higher than those obtained with HPLC/electrochemical detection (ECD). This discrepancy was investigated.

Guidelines for a therapeutic alliance between families and staff: a report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology.

This, the fifth official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, develops another important topic: the Therapeutic Alliance between families and staff. This is addressed to the Pediatric Oncology Community as Guidelines that could be followed. Every parent, medical staff member, and psychosocial professional involved in the care of the child should be responsible for cooperating in the child's best interest.

Dynamics of iron uptake and Fe3O4 biomineralization during aerobic and microaerobic growth of Magnetospirillum gryphiswaldense.

Iron uptake and magnetite (Fe3O4) crystal formation could be studied in the microaerophilic magnetic bacterium Magnetospirillum gryphiswaldense by using a radioactive tracer method for iron transport and a differential light-scattering technique for magnetism. Magnetite formation occurred only in a narrow range of low oxygen concentration, i.e.

Apoptosis and acute lymphocytic leukemia in children.

We examined in vivo spontaneous and prednisolone-induced apoptosis in peripheral blood samples of 23 children with ALL by flow cytometric and morphologic methods. There was no significant spontaneous apoptosis before the therapy. Six hours after prednisolone therapy, increased apoptosis was found in 19 of 23 cases.

Glucocorticoid-induced apoptosis and treatment sensitivity in acute lymphoblastic leukemia of children.

Sensitivity of leukemic blasts to steroid therapy is one of the prognostic factors in acute lymphoblastic leukemia (ALL) in children. We examined the number of steroid receptors and the increase in the apoptotic index in peripheral blast cells after administration of prednisolone monotherapy in 21 children with ALL. A new diagnostic method was established based on determination of the apoptotic index in peripheral blood lymphoblasts to evaluate the steroid sensitivity of leukemic cells during the first day of therapy.

[Chronic granulomatous disease (CGD): dysfunction of the neutrophil granulocyte NADPH-oxidase enzyme system].

Dysfunction of NADPH oxidase results in chronic granulomatous disease (CGD), a syndrome characterized by severe bacterial and fungal infections. Phagocytes of the patients are unable to kill ingested microorganisms which leads to the formation of granulomas and abscesses. Predominant pathogens are the catalase-positive bacteriae (Staphylococcus aureus) and some fungi (Aspergillus species).

Gene expression of neuropeptide Y and neuropeptide-Y1 receptor in relation to proto-oncogene tyrosine kinase A, nerve-growth-factor low-affinity-receptor and the transcription factor N-myc in human neuroblastomas.

The expression of neuropeptide Y (NPY) and NPY-Y1 receptor (NPY-Y1R) in relation to that of tyrosine kinase A (trkA), nerve-growth-factor low-affinity-receptor (LNGFR) and the transcription factor N-myc was studied in 26 neuroblastomas and one ganglioneuroma by quantitative Northern-blot analysis. A correlation of NPY-Y1R with LNGFR (r = 0.85, p < 0.

Iron-limited growth and kinetics of iron uptake in Magnetospirillum gryphiswaldense.

Growth and magnetite formation in Magnetospirillum gryphiswaldense MSR-1 were found close to the maximum at an extracellular iron concentration of 15-20 microM. Ferrous iron was incorporated by a slow, diffusion-like process. Several iron chelators including various microbial siderophores were unable to promote transport of iron into the cells.

[In vivo effect of etoposide on the pharmacokinetics of methotrexate].

High dose (5 g/m2/24 h) methotrexate therapy was combined two times with etoposide (100 mg/m2/1h) infusions as a part of the Medulloblastoma protocol developed in our Department Vepesid therapy was administered in two different schedules. The first group of the patients have received etoposide immediately before and at the end (24th h) of methotrexate treatment. The second group was treated with etoposide at 24 h and at 48 hour after starting methotrexate infusion.

Exploration of interactions between prognostic factors of stage I malignant melanoma.

While many studies have sought prognostic factors of malignant melanoma using multivariate survival models, the interaction between predictors has been much less studied. We have studied data from 1,560 patients with stage I melanoma collected at the Cancer Registry of the Canton of Zurich over the period 1980-1990 and explored interactions between predictors by identifying two separate multivariate Cox models for men and women and investigating two-way interactions between predictors in each model. Considerable differences between models for man and women were observed.

SIOP Working Committee on Psychosocial issues in pediatric oncology: guidelines for care of long-term survivors.

This is the third official document of the SIOP Working Committee on Psychosocial issues in pediatric oncology constituted in 1991. This document develops another topic already discussed and approved by the SIOP committee. The topic: "Care of long-term survivors" is addressed to the pediatric oncology community as guidelines that could be followed for considering this issue in a more appropriate way.

[Immunophenotyping of childhood leukemias--results of the first 100 bone marrow tests].

The authors used a cell-surface-marker analysis by flow cytometry for immunophenotyping of pediatric leukemia from 1993. Results of their first one-hundred cases were summarized. They examined 68 bone-marrow samples from 58 patients affected by leukemia, and diagnosed 19 acute myeloid leukemia (32%), 5 T-cell acute lymphoid leukemia (8%), 32 B-cell acute lymphoid leukemia (54%) and 2 biphenotypic leukemia (5%).

Diffuse plasmacytosis in a child with brainstem glioma following multiagent chemotherapy and intensive growth factor support.

The use of granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor (GM-CSF) in order to abrogate chemotherapy-induced neutropenia has become a routine part of many cancer treatment regimes. However, there are still very few data available about possible complications related to repeated or prolonged use of these agents in patients with malignant solid tumors. The authors report a child with brainstem glioma who received repeated cycles of multiagent chemotherapy with G- or GM-CSF support.

Childhood leukaemia in Europe after Chernobyl: 5 year follow-up.

The European Childhood Leukaemia - Lymphoma Incidence Study (ECLIS) is designed to address concerns about a possible increase in the risk of cancer in Europe following the nuclear accident in Chernobyle in 1986. This paper reports results of surveillance of childhood leukaemia in cancer registry populations from 1980 up to the end of 1991. There was a slight increase in the incidence of childhood leukaemia in Europe during this period, but the overall geographical pattern of change bears no relation to estimated exposure to radiation resulting from the accident.

Crotonaldehyde: a carcinogenic and mutagenic air, water and food pollutant.

Crotonaldehyde is mutagenic and carcinogenic and it is ubiquitous in our environment. The data base does, however, not allow an assessment of the carcinogenic risk. We have developed a sensitive 32P-postlabelling technique which allows the detection of specific DNA-adducts in animal tissues as markers for initiation of cancer cells.

Action profiles of predictors of death and survival time in stage I malignant melanoma.

Background: Many studies have been performed to identify prognostic factors of malignant melanoma using multivariate regression models. In these models, it is generally assumed that quantitative predictors such as age or tumor thickness enter linearly into the model, or they are categorized.

Objective: The purpose of the present study is to investigate possible curvature (nonlinearity) of predictors of 'death from MM within 5 years after diagnosis' and 'survival time after diagnosis' avoiding the known shortcomings of categorizing predictors.

Childhood leukemia and statistical characteristics in 0- to 18-year-old patients in Hungary, diagnosed between 1988 and 1992.

A population-based cancer registry for childhood leukemia was started in Hungary in 1971. Data processing and analysis have been done at the Second Department of Pediatrics, Semmelweis University Medical School in Budapest, which is the main center for the treatment of childhood malignancies in Hungary. In 1992 a new computerized database structure was created in collaboration with Kansas University Medical Center, Kansas City, Kansas, United States.