Katalin Bartyik - Academia.edu (original) (raw)
Papers by Katalin Bartyik
The most common vascular malformation causing respiratory distress in neonates is intralaryngeal ... more The most common vascular malformation causing respiratory distress in neonates is intralaryngeal hemangioma. The authors report rare cases of huge extralaryngeal vascular lesions which caused severe upper airway obstruction requiring intubation right after delivery (CHAOS syndrome). Case 1 is a congenital[for full text, please go to the a.m. URL]
European Journal of Pediatrics, 2001
Colorectal carcinoma rarely aects children and has a dismal prognosis with 5-year survival rates ... more Colorectal carcinoma rarely aects children and has a dismal prognosis with 5-year survival rates as low as 2.5%±7% despite apparently radical surgery. Here we report the case of an adenocarcinoma of the sigmoid colon in a 15-year-old girl preceded by uncertain abdominal complaints of 5 years' duration. Pathological work-up revealed a tumour with lymph node metastases (pT3N1). Immunohistochemical evidence of p53 overexpression by the tumour cells raised the suspicion of an underlying Li-Fraumeni syndrome. In addition, there were aphthoid ulceration, ®ssuration of the non-tumorous mucosa, along with a mixed transmural in®ltrate composed of macrophages, eosinophils, and non-typical giant cells, which were compatible with simultaneous Crohn's disease. Anamnestic data concerning the occurrence of idiopathic in¯ammatory bowel disease or colorectal carcinoma in the patient's relatives were non-contributory. The present results suggest a possible relationship between Crohn's disease and colon cancer due to the defective p53 gene product.
Az egy éves fiúcsecsemőt szülei fájdalmatlan, egészen a kismedencébe leérő hasi terime miatt vitt... more Az egy éves fiúcsecsemőt szülei fájdalmatlan, egészen a kismedencébe leérő hasi terime miatt vitték orvoshoz. A csecsemő korábban egészséges volt, 6 hónaposan bukások miatt történt klinikánkon kivizsgálása gastrooesophagealis reflux irányába. Ekkor történt hasi ultrahang vizsgálat is, mely kórosat nem észlelt a hasban. A családban az édesanya és az anyai nagymama Gardner-szindrómában szenved. Ez a kórkép a familiáris adenomatosus polyposis szindrómák családjába tartozik, ahol az intestinális tünetek mellett extraintestinális, főleg jóindulatú tumorok jelentkezhetnek (osteoid osteoma, dermoid cysta). A betegség öröklödésmenete autoszomális domináns-és genetikai háttere az 5. kromoszómán található APC (adenomatosus polyposis coli) tumor szupresszor gén mutációja. A csecsemő 4 éves fiútestvére egészséges. Fizikális vizsgálattal hatalmas májat tapintottunk, májtumor merült fel.
European Journal of Pediatrics, 2004
Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontr... more Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion. The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.
The aim of this study was to compare the quality of life and school success of adolescent survivo... more The aim of this study was to compare the quality of life and school success of adolescent survivors and their classmates. A survey was conducted among 21 cancer survived 12-18-year-old children and 95 of their classmates by using questionnaires covering (a) characteristics of the quality of life; (b) characteristics of the learning process; and (c) level of the fear of cancer recurrence. Significant difference was found in the field of physical and emotional functions but contrary to expected, the members of the control group reported lower values than survivor children. Those children that were teased because of cancer made friends hardly and got involved in social programs with more difficulty. With reference to the level of development of school motivation and the use of learning strategies, it was experienced a significant difference between the two groups only in the field of planning. Our results show that the better the survived children's general quality of life is the better results they achieve at school. Their learning achievement is influenced to a much bigger extent by social functions than their physical disadvantages.
Introduction 12 Chapterl-Diagnostic problems 16 1.1. Pulmonary capillary haemangiomatosis in chil... more Introduction 12 Chapterl-Diagnostic problems 16 1.1. Pulmonary capillary haemangiomatosis in children and adolescents 16 1.2. Erythema nodosum associated with coeliac disease in a teenage girl 18 with cured ALL and haemangioblastoma 1.3 Adenocarcinoma of the colon developing on the basis of Crohn's disease in childhood 1.4 Hepatoblastoma in a family with Gardner syndrome 1.5 Paraneoplastic erythrocytosis in Wilms tumour Chapter2-Therapeutic issues 26 2.1. Good response for a new therapy in a childhood CML 26 2.2. Refractory B-cell lymphoma treated successfully with rituximab and autologous stem cell transplant 2.3. Immunization against varicella of children receiving cytostatic, 30 immunosuppessive treatment. A historical perspective 2.4. Successful treatment of Aspergillus infection in a child with (secondary) malignancy 2.5 Toxicity of high-dose methotrexate treatment 2.6 Late effects on renal function in childhood cancer survivors Chapter3-Biochemical topics 3.1 Activity of cathepsin B, H, L and MMP7 in serum of children with ALL and tumours 3.2. Methotrexate inhibits the glyoxalase system in vivo in children with ALL References
Detection of Residual Tumor Load and Pre-leukemic Cell Clone Using Combined Phenoand Genotyping a... more Detection of Residual Tumor Load and Pre-leukemic Cell Clone Using Combined Phenoand Genotyping at Single Cell Level Donat Alpar1, Renata Laszlo1, Eszter Benko1, Bela Kajtar1, Agnes Lacza1, Gabor Ottoffy2, Katalin Bartyik3, Kalman Nagy4, Laszlo Pajor1 1Department of Pathology, University of Pecs, Pecs, HU7624, Hungary, 2Department of Pediatric Oncology, University of Pecs, Pecs, HU-7624, Hungary, 3Department of Pediatric Oncology, University of Szeged, Szeged, Hungary, 4Department of Hematology, Borsod County Teaching Hospital, Miskolc, Hungary
Using combined chemotherapy, pediatric ALL (acute lymphoblastic leukemia) is a very curable disea... more Using combined chemotherapy, pediatric ALL (acute lymphoblastic leukemia) is a very curable disease. In Hungary, approximately 85% of patients with ALL survive 5 years after therapy [1]. The Berlin–Frankfurt–Münster (BFM) group first used the nucleoside analogue cytarabine (cytosine arabinoside, 1-b-Darabinofuranosylcytosine, ara-C) in 1981 in combination with methotrexate, cyclophosphamide and doxorubicin [2–4]. However, the therapeutic agents used in the treatment of this disease are highly toxic and induce serious side effects. The major toxicities of ara-C at standard doses are myelosuppression, mucositis and infection [5]. Cytopenias as the result of myelosuppression can rapidly become life threatening or affect the quality of life, often leading to interruptions in chemotherapy and a subsequent increase in the risk of relapse. Because there is a high interpatient variability of sensitivity and toxicity to ara-C, understanding the background of this variance could provide an op...
Orvosi hetilap, 2005
BACKGROUND In Hungary children (from 1 to 18 years of age) with de novo acute lymphoblastic leuke... more BACKGROUND In Hungary children (from 1 to 18 years of age) with de novo acute lymphoblastic leukemia were treated from January 1996 to October 2002, according to protocol ALL-BFM-95. AIM The aim of this study was to evaluate the experience with this protocol, the treatment results according to the risk groups and to compare the Hungarian data with the international results. METHODS Patients were stratified into 3 risk groups, based on initial white blood cell count, age, immunology, cytogenetics and response to treatment: standard, medium and high risk group. RESULTS Three hundred sixty eight children entered the study (male-female ratio was 1.27:1, median age 6 years and 4 months). 110 (29.9%) children were in the standard, 210 (57.1%) in the medium and 48 (13%) in the high risk group. Duration of the chemotherapy was 2 years, except of the boys in the standard risk group, their maintenance therapy was 1 year longer. The overall complete remission rate was 93.2%. 20 (5.4%) children...
Scientific Reports
the Hungarian pediatric oncology network provides centralized treatment and population-based regi... more the Hungarian pediatric oncology network provides centralized treatment and population-based registration for cases of childhood cancer since 1973. We collected and analized data on late mortality, secondary malignancies and cardiac diseases in survivors (> 5 years) of childhood cancer to evaluate long-term risks. We extracted all solid tumour cases (3,650 followed up for 5-39.3 years, diagnosis: 1973-2008) from the database of the Hungarian Childhood Cancer Registry and checked against the Population Registry. Among the 301 patients who died after 5 years (8.2%) the most common causes of death were progression of primary cancer (52.5%), secondary malignancies (16%) and cardiovascular diseases (8%). Late mortality rates (SMR, total: 35,006 pyrs) showed highly elevated risk of death (SMR: 10.7 95% CI 9-12.4) for the second 5 years of follow up and moderately elevated risk for 10-year survivors (SMR: 3.5 95% CI 3-4.1). Marked differences were detected in the pattern of causes of death between diagnostic groups of primary cancer; with highest risks beyond 10 years for CNS tumours, Hodgkin disease, osteosarcoma and advanced stage neuroblastoma. The longstanding mortality risk for 5-year survivors underlines the need for tailored long-term follow-up and monitoring of late consequences according to the context of different primary diseases of childhood cancer. Centralised treatment protocols and national registration for children with leukemia and solid tumours was introduced by centres of the Hungarian Pediatric Oncology Network (HPON) from 1971. Concomitantly, the Hungarian Childhood Cancer Registry (HCCR) was founded. It represents one of the oldest childhood cancer registries in Europe providing population-based registration of incidence and mortality (< 15 years). Definitive diagnosis and treatment of malignant diseases was uniformly based on consensus protocols agreed by HPON. Detailed data were collected on all cases including benign CNS (central nervous system) tumours on the basis of compulsory registration including wide range of variables including primary tumour sites, histologic and prognostic markers, details of treatment and outcome, relapses, early and late toxicities and long-term follow-up 1-3. Outcome of childhood cancer has shown great improvement from the nineteen sixties due to progress in multimodal therapy 4-6. Survival rates of different diseases exceeded 50% at different time periods during the last 50 years 7,8. As outcome of diseases showed improvement, the average survival time of patients increased. Decreasing relapse risk and improving second line therapy led to longer survival 7,8. Consequently, the number of long-term survivors has increased corresponding to one in 870 adolescents entering adulthood in Hungary. Why do late events happen? Either disease biology or therapeutic complications or both can be responsible. Children show high sensitivity to side effects of chemo/radiotherapy which can lead to severe complex problems, sometimes fatal events. In as few as 5-10 years an elevated risk of secondary malignancies can be detected, showing increasing trend in time with no plateau formation 9,10. In general, one third of survivors are free of late effects, but two thirds have detectable organ damage, half of them severe and/or combined late
Journal of Cancer Education
The aim of this study was to compare the quality of life and school success of adolescent survivo... more The aim of this study was to compare the quality of life and school success of adolescent survivors and their classmates. A survey was conducted among 21 cancer survived 12-18-year-old children and 95 of their classmates by using questionnaires covering (a) characteristics of the quality of life; (b) characteristics of the learning process; and (c) level of the fear of cancer recurrence. Significant difference was found in the field of physical and emotional functions but contrary to expected, the members of the control group reported lower values than survivor children. Those children that were teased because of cancer made friends hardly and got involved in social programs with more difficulty. With reference to the level of development of school motivation and the use of learning strategies, it was experienced a significant difference between the two groups only in the field of planning. Our results show that the better the survived children's general quality of life is the better results they achieve at school. Their learning achievement is influenced to a much bigger extent by social functions than their physical disadvantages.
![Research paper thumbnail of [Change in paradigm in the treatment of pediatric acquired bone marrow failure syndromes in Hungary]](https://attachments.academia-assets.com/71625602/thumbnails/1.jpg)
](Orvosi hetilap, 2018
Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has b... more Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has been participating as a full member in the work of the European Working Group on uniform diagnostics and therapy in patients with acquired bone marrow failure syndromes. Hypocellular refractory cytopenia of childhood has been emphasized as a frequent entity, transplanted by reduced intensity conditioning with excellent outcomes. To analyse and compare the results of treatment before and after our joining. A total of 55 patients have been treated in the 8 centres of the Hungarian Pediatric Oncology Network during 5 years between 2013 and 2017 (severe aplastic anemia: 9, myelodysplastic syndrome: 41, juvenile myelomonocytic leukemia: 5 patients). Allogeneic hematopoietic stem cell transplantation was performed in severe aplastic anemia in 7 cases, while antithymocyte globulin was administered in one case and one patient died before diagnosis. In patients with myelodysplastic syndromes, watc...
Pathology Oncology Research Por, Feb 1, 2007
To prevent acute renal failure in children at risk for developing tumor lysis syndrome due to acu... more To prevent acute renal failure in children at risk for developing tumor lysis syndrome due to acute lymphoblastic leukemia or non-Hodgkin's lymphoma treated according to international BFM protocols, we investigated recombinant urate oxidase (rasburicase) in the first Central European openlabeled, prospective, multicenter phase IV trial. Rasburicase was administered intravenously, at 0.2 mg/kg for 5 consecutive days to 36 patients. Blood levels of uric acid, creatinine, phosphorus, calcium, lactate dehydrogenase and complete blood count were measured daily during rasburicase treatment and on days 6, 7 and 12. Initial uric acid level decreased significantly by 4 hours (from 343 µmol/L to 58 µmol/L, p<0.001), except for one steroid-resistant patient who required hemodialysis on day 14 after having introduced combined cytostatic treatment. Comparing the data of a subgroup of 12 patients receiving rasburicase with that of a historic cohort of 14 patients treated with allopurinol indicated the superiority of rasburicase over allopurinol in prophylaxis and treatment of hyperuricemia in children with leukemia and lymphoma.
Journal of neuro-oncology, Jan 15, 2015
The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant varia... more The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant variant of embryonal brain tumors. It usually affects infants and young children under the age of 4 years and exhibits a very aggressive course with a dismal prognosis. For the 68 cases reported to date the mean age at diagnosis was 25.42 months (range 3-57 months). Survival data are available for 48 children (including our case): the median overall survival is 13.0 months, though 6 (9 %) of the children have had a relative long survival (>30 months). The aggressive combined treatment, involving primary surgical tumor removal, adjuvant polychemotherapy, including high-dose chemotherapy with stem cell transplantation, radiotherapy and radiochemotherapy, might play an important role in the longer survival. We have performed a literature review and we present here a multimodal-treated case of a 2- year-old girl with a long survival, who was reoperated when recurrence occurred. The residual tu...
Pathology oncology research : POR, 2013
The aetiology of childhood acute lymphoblastic leukaemia has been linked with spatially heterogen... more The aetiology of childhood acute lymphoblastic leukaemia has been linked with spatially heterogeneous environmental exposures. The presence of spatial clustering would be consistent with geographically localized environmental exposures over long periods of time. The present study is the first to examine spatial clustering amongst children aged 0-4 years using population-based data from Hungary. The data set consisted of 134 children diagnosed with acute lymphoblastic leukaemia who were resident in part of Hungary during the period 1981-2000. Two levels of spatial aggregation were examined: counties and settlements. The Potthoff-Whittinghill and Moran I autocorrelation methods were used to test for spatial clustering. Additionally, an evaluation of the environmental changes during the study period was considered. Specifically analyses were carried out on sub-periods to investigate a possible effect of the Chernobyl catastrophe. There was statistically significant spatial clustering b...
International Journal of Pediatric Otorhinolaryngology, 2014
Neuroblastoma is the most common malignancy in infancy, it is a histologically and genetically he... more Neuroblastoma is the most common malignancy in infancy, it is a histologically and genetically heterogeneous tumor, the therapy and outcome of which is influenced by age, histological variant and genetic background as well. We present two consecutive infant patients with neuroblastoma of the neck discussing the etiology, the diagnosis and the surgical and oncological treatment of the tumor, which was observed in a relatively rare manifestation in the head-neck region. Our first patient (age: 5.5 months) was MYCN (v-myc myelocytomatosis viral related oncogene, neuroblastoma derived) negative, INSS (International Neuroblastoma Staging System) Stage 3 and INRGSS (International Neuroblastoma Risk Group Staging System) Stage 3 because of the contralateral lymph node involvement while the complete gross resection of the primary tumor mass was feasible. The patient is tumor free after three years of follow-up. Our second patient (age: 5 months) was MYCN negative, INSS Stage 2 and INRGSS Stage 1, as both the primary tumor and the ipsilateral lymph nodes were totally removed via a modified radical neck dissection. The patient is tumor free after three years of follow-up. For MYCN negative patients, especially in early age, the prognosis of neuroblastoma is good, surgical resection and chemotherapy together is an adequate treatment protocol (as in our two patients). While MYCN-amplified patients require a combined and aggressive treatment with surgery, chemotherapy, radiotherapy, and immunotherapy to be able to obtain a favorable survival rate according to the literature.
The most common vascular malformation causing respiratory distress in neonates is intralaryngeal ... more The most common vascular malformation causing respiratory distress in neonates is intralaryngeal hemangioma. The authors report rare cases of huge extralaryngeal vascular lesions which caused severe upper airway obstruction requiring intubation right after delivery (CHAOS syndrome). Case 1 is a congenital[for full text, please go to the a.m. URL]
European Journal of Pediatrics, 2001
Colorectal carcinoma rarely aects children and has a dismal prognosis with 5-year survival rates ... more Colorectal carcinoma rarely aects children and has a dismal prognosis with 5-year survival rates as low as 2.5%±7% despite apparently radical surgery. Here we report the case of an adenocarcinoma of the sigmoid colon in a 15-year-old girl preceded by uncertain abdominal complaints of 5 years' duration. Pathological work-up revealed a tumour with lymph node metastases (pT3N1). Immunohistochemical evidence of p53 overexpression by the tumour cells raised the suspicion of an underlying Li-Fraumeni syndrome. In addition, there were aphthoid ulceration, ®ssuration of the non-tumorous mucosa, along with a mixed transmural in®ltrate composed of macrophages, eosinophils, and non-typical giant cells, which were compatible with simultaneous Crohn's disease. Anamnestic data concerning the occurrence of idiopathic in¯ammatory bowel disease or colorectal carcinoma in the patient's relatives were non-contributory. The present results suggest a possible relationship between Crohn's disease and colon cancer due to the defective p53 gene product.
Az egy éves fiúcsecsemőt szülei fájdalmatlan, egészen a kismedencébe leérő hasi terime miatt vitt... more Az egy éves fiúcsecsemőt szülei fájdalmatlan, egészen a kismedencébe leérő hasi terime miatt vitték orvoshoz. A csecsemő korábban egészséges volt, 6 hónaposan bukások miatt történt klinikánkon kivizsgálása gastrooesophagealis reflux irányába. Ekkor történt hasi ultrahang vizsgálat is, mely kórosat nem észlelt a hasban. A családban az édesanya és az anyai nagymama Gardner-szindrómában szenved. Ez a kórkép a familiáris adenomatosus polyposis szindrómák családjába tartozik, ahol az intestinális tünetek mellett extraintestinális, főleg jóindulatú tumorok jelentkezhetnek (osteoid osteoma, dermoid cysta). A betegség öröklödésmenete autoszomális domináns-és genetikai háttere az 5. kromoszómán található APC (adenomatosus polyposis coli) tumor szupresszor gén mutációja. A csecsemő 4 éves fiútestvére egészséges. Fizikális vizsgálattal hatalmas májat tapintottunk, májtumor merült fel.
European Journal of Pediatrics, 2004
Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontr... more Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion. The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.
The aim of this study was to compare the quality of life and school success of adolescent survivo... more The aim of this study was to compare the quality of life and school success of adolescent survivors and their classmates. A survey was conducted among 21 cancer survived 12-18-year-old children and 95 of their classmates by using questionnaires covering (a) characteristics of the quality of life; (b) characteristics of the learning process; and (c) level of the fear of cancer recurrence. Significant difference was found in the field of physical and emotional functions but contrary to expected, the members of the control group reported lower values than survivor children. Those children that were teased because of cancer made friends hardly and got involved in social programs with more difficulty. With reference to the level of development of school motivation and the use of learning strategies, it was experienced a significant difference between the two groups only in the field of planning. Our results show that the better the survived children's general quality of life is the better results they achieve at school. Their learning achievement is influenced to a much bigger extent by social functions than their physical disadvantages.
Introduction 12 Chapterl-Diagnostic problems 16 1.1. Pulmonary capillary haemangiomatosis in chil... more Introduction 12 Chapterl-Diagnostic problems 16 1.1. Pulmonary capillary haemangiomatosis in children and adolescents 16 1.2. Erythema nodosum associated with coeliac disease in a teenage girl 18 with cured ALL and haemangioblastoma 1.3 Adenocarcinoma of the colon developing on the basis of Crohn's disease in childhood 1.4 Hepatoblastoma in a family with Gardner syndrome 1.5 Paraneoplastic erythrocytosis in Wilms tumour Chapter2-Therapeutic issues 26 2.1. Good response for a new therapy in a childhood CML 26 2.2. Refractory B-cell lymphoma treated successfully with rituximab and autologous stem cell transplant 2.3. Immunization against varicella of children receiving cytostatic, 30 immunosuppessive treatment. A historical perspective 2.4. Successful treatment of Aspergillus infection in a child with (secondary) malignancy 2.5 Toxicity of high-dose methotrexate treatment 2.6 Late effects on renal function in childhood cancer survivors Chapter3-Biochemical topics 3.1 Activity of cathepsin B, H, L and MMP7 in serum of children with ALL and tumours 3.2. Methotrexate inhibits the glyoxalase system in vivo in children with ALL References
Detection of Residual Tumor Load and Pre-leukemic Cell Clone Using Combined Phenoand Genotyping a... more Detection of Residual Tumor Load and Pre-leukemic Cell Clone Using Combined Phenoand Genotyping at Single Cell Level Donat Alpar1, Renata Laszlo1, Eszter Benko1, Bela Kajtar1, Agnes Lacza1, Gabor Ottoffy2, Katalin Bartyik3, Kalman Nagy4, Laszlo Pajor1 1Department of Pathology, University of Pecs, Pecs, HU7624, Hungary, 2Department of Pediatric Oncology, University of Pecs, Pecs, HU-7624, Hungary, 3Department of Pediatric Oncology, University of Szeged, Szeged, Hungary, 4Department of Hematology, Borsod County Teaching Hospital, Miskolc, Hungary
Using combined chemotherapy, pediatric ALL (acute lymphoblastic leukemia) is a very curable disea... more Using combined chemotherapy, pediatric ALL (acute lymphoblastic leukemia) is a very curable disease. In Hungary, approximately 85% of patients with ALL survive 5 years after therapy [1]. The Berlin–Frankfurt–Münster (BFM) group first used the nucleoside analogue cytarabine (cytosine arabinoside, 1-b-Darabinofuranosylcytosine, ara-C) in 1981 in combination with methotrexate, cyclophosphamide and doxorubicin [2–4]. However, the therapeutic agents used in the treatment of this disease are highly toxic and induce serious side effects. The major toxicities of ara-C at standard doses are myelosuppression, mucositis and infection [5]. Cytopenias as the result of myelosuppression can rapidly become life threatening or affect the quality of life, often leading to interruptions in chemotherapy and a subsequent increase in the risk of relapse. Because there is a high interpatient variability of sensitivity and toxicity to ara-C, understanding the background of this variance could provide an op...
Orvosi hetilap, 2005
BACKGROUND In Hungary children (from 1 to 18 years of age) with de novo acute lymphoblastic leuke... more BACKGROUND In Hungary children (from 1 to 18 years of age) with de novo acute lymphoblastic leukemia were treated from January 1996 to October 2002, according to protocol ALL-BFM-95. AIM The aim of this study was to evaluate the experience with this protocol, the treatment results according to the risk groups and to compare the Hungarian data with the international results. METHODS Patients were stratified into 3 risk groups, based on initial white blood cell count, age, immunology, cytogenetics and response to treatment: standard, medium and high risk group. RESULTS Three hundred sixty eight children entered the study (male-female ratio was 1.27:1, median age 6 years and 4 months). 110 (29.9%) children were in the standard, 210 (57.1%) in the medium and 48 (13%) in the high risk group. Duration of the chemotherapy was 2 years, except of the boys in the standard risk group, their maintenance therapy was 1 year longer. The overall complete remission rate was 93.2%. 20 (5.4%) children...
Scientific Reports
the Hungarian pediatric oncology network provides centralized treatment and population-based regi... more the Hungarian pediatric oncology network provides centralized treatment and population-based registration for cases of childhood cancer since 1973. We collected and analized data on late mortality, secondary malignancies and cardiac diseases in survivors (> 5 years) of childhood cancer to evaluate long-term risks. We extracted all solid tumour cases (3,650 followed up for 5-39.3 years, diagnosis: 1973-2008) from the database of the Hungarian Childhood Cancer Registry and checked against the Population Registry. Among the 301 patients who died after 5 years (8.2%) the most common causes of death were progression of primary cancer (52.5%), secondary malignancies (16%) and cardiovascular diseases (8%). Late mortality rates (SMR, total: 35,006 pyrs) showed highly elevated risk of death (SMR: 10.7 95% CI 9-12.4) for the second 5 years of follow up and moderately elevated risk for 10-year survivors (SMR: 3.5 95% CI 3-4.1). Marked differences were detected in the pattern of causes of death between diagnostic groups of primary cancer; with highest risks beyond 10 years for CNS tumours, Hodgkin disease, osteosarcoma and advanced stage neuroblastoma. The longstanding mortality risk for 5-year survivors underlines the need for tailored long-term follow-up and monitoring of late consequences according to the context of different primary diseases of childhood cancer. Centralised treatment protocols and national registration for children with leukemia and solid tumours was introduced by centres of the Hungarian Pediatric Oncology Network (HPON) from 1971. Concomitantly, the Hungarian Childhood Cancer Registry (HCCR) was founded. It represents one of the oldest childhood cancer registries in Europe providing population-based registration of incidence and mortality (< 15 years). Definitive diagnosis and treatment of malignant diseases was uniformly based on consensus protocols agreed by HPON. Detailed data were collected on all cases including benign CNS (central nervous system) tumours on the basis of compulsory registration including wide range of variables including primary tumour sites, histologic and prognostic markers, details of treatment and outcome, relapses, early and late toxicities and long-term follow-up 1-3. Outcome of childhood cancer has shown great improvement from the nineteen sixties due to progress in multimodal therapy 4-6. Survival rates of different diseases exceeded 50% at different time periods during the last 50 years 7,8. As outcome of diseases showed improvement, the average survival time of patients increased. Decreasing relapse risk and improving second line therapy led to longer survival 7,8. Consequently, the number of long-term survivors has increased corresponding to one in 870 adolescents entering adulthood in Hungary. Why do late events happen? Either disease biology or therapeutic complications or both can be responsible. Children show high sensitivity to side effects of chemo/radiotherapy which can lead to severe complex problems, sometimes fatal events. In as few as 5-10 years an elevated risk of secondary malignancies can be detected, showing increasing trend in time with no plateau formation 9,10. In general, one third of survivors are free of late effects, but two thirds have detectable organ damage, half of them severe and/or combined late
Journal of Cancer Education
The aim of this study was to compare the quality of life and school success of adolescent survivo... more The aim of this study was to compare the quality of life and school success of adolescent survivors and their classmates. A survey was conducted among 21 cancer survived 12-18-year-old children and 95 of their classmates by using questionnaires covering (a) characteristics of the quality of life; (b) characteristics of the learning process; and (c) level of the fear of cancer recurrence. Significant difference was found in the field of physical and emotional functions but contrary to expected, the members of the control group reported lower values than survivor children. Those children that were teased because of cancer made friends hardly and got involved in social programs with more difficulty. With reference to the level of development of school motivation and the use of learning strategies, it was experienced a significant difference between the two groups only in the field of planning. Our results show that the better the survived children's general quality of life is the better results they achieve at school. Their learning achievement is influenced to a much bigger extent by social functions than their physical disadvantages.
Orvosi hetilap, 2018
Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has b... more Acquired bone marrow failures are rare but fatal diseases in childhood. Since 2013, Hungary has been participating as a full member in the work of the European Working Group on uniform diagnostics and therapy in patients with acquired bone marrow failure syndromes. Hypocellular refractory cytopenia of childhood has been emphasized as a frequent entity, transplanted by reduced intensity conditioning with excellent outcomes. To analyse and compare the results of treatment before and after our joining. A total of 55 patients have been treated in the 8 centres of the Hungarian Pediatric Oncology Network during 5 years between 2013 and 2017 (severe aplastic anemia: 9, myelodysplastic syndrome: 41, juvenile myelomonocytic leukemia: 5 patients). Allogeneic hematopoietic stem cell transplantation was performed in severe aplastic anemia in 7 cases, while antithymocyte globulin was administered in one case and one patient died before diagnosis. In patients with myelodysplastic syndromes, watc...
Pathology Oncology Research Por, Feb 1, 2007
To prevent acute renal failure in children at risk for developing tumor lysis syndrome due to acu... more To prevent acute renal failure in children at risk for developing tumor lysis syndrome due to acute lymphoblastic leukemia or non-Hodgkin's lymphoma treated according to international BFM protocols, we investigated recombinant urate oxidase (rasburicase) in the first Central European openlabeled, prospective, multicenter phase IV trial. Rasburicase was administered intravenously, at 0.2 mg/kg for 5 consecutive days to 36 patients. Blood levels of uric acid, creatinine, phosphorus, calcium, lactate dehydrogenase and complete blood count were measured daily during rasburicase treatment and on days 6, 7 and 12. Initial uric acid level decreased significantly by 4 hours (from 343 µmol/L to 58 µmol/L, p<0.001), except for one steroid-resistant patient who required hemodialysis on day 14 after having introduced combined cytostatic treatment. Comparing the data of a subgroup of 12 patients receiving rasburicase with that of a historic cohort of 14 patients treated with allopurinol indicated the superiority of rasburicase over allopurinol in prophylaxis and treatment of hyperuricemia in children with leukemia and lymphoma.
Journal of neuro-oncology, Jan 15, 2015
The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant varia... more The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant variant of embryonal brain tumors. It usually affects infants and young children under the age of 4 years and exhibits a very aggressive course with a dismal prognosis. For the 68 cases reported to date the mean age at diagnosis was 25.42 months (range 3-57 months). Survival data are available for 48 children (including our case): the median overall survival is 13.0 months, though 6 (9 %) of the children have had a relative long survival (>30 months). The aggressive combined treatment, involving primary surgical tumor removal, adjuvant polychemotherapy, including high-dose chemotherapy with stem cell transplantation, radiotherapy and radiochemotherapy, might play an important role in the longer survival. We have performed a literature review and we present here a multimodal-treated case of a 2- year-old girl with a long survival, who was reoperated when recurrence occurred. The residual tu...
Pathology oncology research : POR, 2013
The aetiology of childhood acute lymphoblastic leukaemia has been linked with spatially heterogen... more The aetiology of childhood acute lymphoblastic leukaemia has been linked with spatially heterogeneous environmental exposures. The presence of spatial clustering would be consistent with geographically localized environmental exposures over long periods of time. The present study is the first to examine spatial clustering amongst children aged 0-4 years using population-based data from Hungary. The data set consisted of 134 children diagnosed with acute lymphoblastic leukaemia who were resident in part of Hungary during the period 1981-2000. Two levels of spatial aggregation were examined: counties and settlements. The Potthoff-Whittinghill and Moran I autocorrelation methods were used to test for spatial clustering. Additionally, an evaluation of the environmental changes during the study period was considered. Specifically analyses were carried out on sub-periods to investigate a possible effect of the Chernobyl catastrophe. There was statistically significant spatial clustering b...
International Journal of Pediatric Otorhinolaryngology, 2014
Neuroblastoma is the most common malignancy in infancy, it is a histologically and genetically he... more Neuroblastoma is the most common malignancy in infancy, it is a histologically and genetically heterogeneous tumor, the therapy and outcome of which is influenced by age, histological variant and genetic background as well. We present two consecutive infant patients with neuroblastoma of the neck discussing the etiology, the diagnosis and the surgical and oncological treatment of the tumor, which was observed in a relatively rare manifestation in the head-neck region. Our first patient (age: 5.5 months) was MYCN (v-myc myelocytomatosis viral related oncogene, neuroblastoma derived) negative, INSS (International Neuroblastoma Staging System) Stage 3 and INRGSS (International Neuroblastoma Risk Group Staging System) Stage 3 because of the contralateral lymph node involvement while the complete gross resection of the primary tumor mass was feasible. The patient is tumor free after three years of follow-up. Our second patient (age: 5 months) was MYCN negative, INSS Stage 2 and INRGSS Stage 1, as both the primary tumor and the ipsilateral lymph nodes were totally removed via a modified radical neck dissection. The patient is tumor free after three years of follow-up. For MYCN negative patients, especially in early age, the prognosis of neuroblastoma is good, surgical resection and chemotherapy together is an adequate treatment protocol (as in our two patients). While MYCN-amplified patients require a combined and aggressive treatment with surgery, chemotherapy, radiotherapy, and immunotherapy to be able to obtain a favorable survival rate according to the literature.