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Biomedical subjects

R Koós

Publications and source records attributed to R Koós.

At least 19 recordsLinked to original sources

[Treatment results of childhood Hodgkin's lymphoma in Hungary]

Lymphomas are the third most frequent malignancies in childhood. The Hungarian Pediatric Oncology Group was founded in 1971, and since then the same chemotherapeutic protocols have been used in the whole country. In this study we analyzed the data of childhood Hodgkin's lymphoma in Hungary in the last 11 years (1988-1998). We also compared our results with the international (German) data. The incidence of Hodgkin's lymphoma (0-15 years) was 7.1/1,000,000 child/year (the same for non-Hodgkin's lymphoma was 7.5/1,000,000/year); 5.5% of all pediatric malignancies in Hungary). The patients were treated according to the German DAL-HD-82 and 90 protocols. The therapy consisted of 2-6 cytostatic blocks, depending on the stage, followed by involved field irradiation. The overall survival was 94.7+/-2.0% at 5 years and 91.9+/-2.7% at 10 years. These results are very similar to the German data: 94% at 5 years and 93% at 10 years. The good results are due to the well organised network and the uniformed treatment. The results may be ameliorated by using autologous bone marrow transplantation.

Journal Article↗

[Results of limb-saving surgery and prognostic factors in patients with osteosarcoma].

Ninety six patients with high-grade osteosarcoma of the extremities were treated between 1986 and 1997 in the authors institution. They were divided into three groups: in group I, all of 75 patients with non-metastatic OS received intensive chemotherapy and underwent surgery. In group II, 9 patients already had metastases at the time of referral. In group III, 12 patients received chemotherapy in delayed or suboptimal form. In group I, local recurrences occurred in 7 per cent (3 patients), metastases in 20 per cent of the patients with limb-saving, whereas these numbers were 3 per cent and 38 per cent in the amputation group. The 5-year disease free survival (DFS) was 72% v 69% in the limb-saving and amputation group, respectively. In groups II and III, 5-year DFS was extremely poor, 10 and 20% only. With univariate analysis, factors having a positive influence on the survival were: tumor volume < 60 cm3, wide or radical surgical margin, distal location of osteosarcoma, cartilagineous ground substance less than 20% and response to chemotherapy. The last 4 variables maintained their significance in the multivariate Cox model as well. Age > 30 showed indirect negative influence on the final outcome (enhanced intolerability to the drugs and less co-operability of the patients etc.). This data confirm the competence of the limb-saving surgery at certain indications beside the amputation.

Adolescent↗

Limb-saving surgery, survival, and prognostic factors for osteosarcoma: the Hungarian experience.

BACKGROUND AND OBJECTIVES: There are many factors thought to have an influence on the prognosis of osteosarcoma that have been reported in the literature. Their significance, however, still remains controversial in most cases. Experience with osteogenic sarcoma (OS) was reviewed in order to evaluate surgical results and survival and to determine the prognostic factors. METHODS: Ninety-six patients with high-grade osteosarcoma of the extremities were treated between 1986 and 1997 in the authors' institution. They were divided into 3 groups: In group I, all 75 patients with nonmetastatic OS received intensive chemotherapy (high-dose methotrexate, doxorubicin, ifosfamide, and cisplatin) and underwent surgery. In group II, 9 patients already had metastases at the time of referral. In group III, 12 patients received chemotherapy in delayed or suboptimal form. Results and Conclusions In group I, there were local recurrences in 3 patients (7%) and metastases in 8 patients (20%) with limb-saving, whereas these numbers were 1 (3%) and 14 (38%) in those who had amputation. The 5-year disease-free survival (DFS) was 72% and 69% in the limb-saving and amputation groups, respectively. In groups II and III, 5-year DFS was extremely poor, 10% and 20% only, underlining the importance of stage and intensity of chemotherapy, respectively. With univariate analysis, sex, duration of symptoms, and radiographic appearance of OS had no prognostic value, whereas tumor volume <60 cm(3), wide or radical surgical margin, distal location of OS, cartilagineous ground substance <20%, and response to chemotherapy were positive prognostic factors. The last 4 variables maintained their significance in the multivariate Cox model as well. Age >30 years showed indirect negative influence on the final outcome through enhanced intolerability to the drugs and less cooperability of the patients. The results on survival with limb-saving surgery were well comparable with those of amputation.

Adolescent↗

Acid sphingomyelinase deficiency in Beckwith Wiedemann syndrome.

We report the association of Beckwith-Wiedemann syndrome (BWS) and a residual acid sphingomyelinase (ASM) activity of about 35% in a 23 months old Hungarian boy. Besides the classical triad of exomphalos, macroglossia and gigantism some other BWS-related features: polyhydramnios (known from the praenatal history), hemihypertrophy, craniofacial dysmorphy, a mild mental retardation, bilaterally undescended testes, cardiac anomalies and a terminally developed, fatal embryonal rhabdomyosarcoma were present in the patient. The decreased activity of the ASM was measured in the patient s skin fibroblasts. This result, with hepatomegaly, mental retardation, feeding problems, a failure to thrive and muscle-hypotony, partially resembled the ASM-deficient forms of Niemann-Pick disease (NPD). Morphological analysis of the bone-marrow cells gave normal results. There was no chromosomal alteration found by conventional karyotyping of the patient s lymphocytes.BWS-associated genes as well as the human ASM gene (SMPD1) are all located at 11p15. DNA-studies by region specific markers as well as mutational analysis for the most common NPD-mutations are planned in the future. This is the first report on the simultaneous occurrence of BWS and ASM-deficiency.

Beckwith-Wiedemann Syndrome↗

[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%). A higher CD34 antigen positivity was found compared to adults. They analysed the incidence and prognostic significance of myeloid + lymphoid leukemia and lymphoid + myeloid leukemia cases. Nondiagnostic and unsuccessful cases were got according to the literature.

Adolescent↗

[First experience with the use of granulocyte-macrophage colony stimulating factor in pediatric oncology].

Authors report their first experiences with the application of granulocyte-macrophage colony stimulating factor in 12 pediatric cancer patients (14 cases). The drug was given in a 5 micrograms/kg single daily dose subcutaneously. Patients were divided into three main indication groups: 1. Severe neutropenia (white blood cell count < 1.0 G/l) and sepsis (6 patients); 2. Prolonged neutropenia (white blood cell count: 1.0-2.0 G/l) and delay in treatment (3 patients); 3. Dose-escalation of chemotherapy in therapy-resistant cases (4 patients). Authors report that in all cases a substantial raise in white blood cell count could be achieved after 5-6 days of granulocyte-macrophage colony stimulating factor treatment. No side effects were detected except of a moderate local pain at the site of the injection. Authors suggest that in the above described dose and way of administration granulocyte-macrophage colony stimulating factor can be an effective agent in the treatment of chemotherapy-induced neutropenia in paediatric oncology.

Adolescent↗

[Use of ondansetron, a 5-HT3 receptor antagonist, as a new type of antiemetic in pediatric oncology].

The effectiveness of the new antiemetic drug, the 5-hydroxytryptamin (5-HT) receptor antagonist ondansetron was evaluated in paediatric cancer patients. 5-HT3 antagonists represent a new class of drugs effective in the control of chemo- and radiotherapy-induced emesis. Based on their selectivity 5-HT3 antagonist are free from extrapyramidal side effects, a major problem in children in the case of currently used dopamine receptor antagonists (e.g. metoclopramide). In this study ondansetron was tested as antiemetic in 33 children with malignant disease (132 chemotherapy cycles) treated with: 1. high-dose cisplatin (120 mg/m2), 2. intermediate-dose cisplatin (60 mg/m2) and 3. no cisplatin-containing, combined high-dose chemotherapy. Ondansetron was found to be safe and effective in the control of acute and delayed emesis in all treatment groups. Its effectiveness was superior to the currently used antiemetic drugs in the period of acute emesis.

Adolescent↗

The treatment of malignant scala posterior tumors in children: II. Preliminary result of the pre- and postoperative adjuvant chemotherapy of scala posterior tumors.

Ten children with posterior scala tumor infiltrating the surrounding brain substance and/or the brain stem entered in the present study with preoperative chemotherapy. In 8 of the 10 cases regression and necrosis of the tumor were seen by CT examination after the preoperative therapy. The diameter of the tumor decreased on the average by 35.6% (14.0-74.3%). The main side effect was granulocytopenia. According to our observation, the preoperative therapy enables a more radical surgery in some cases of medulloblastoma and ependymoma. Further observations are necessary to confirm these preliminary results.

Antineoplastic Combined Chemotherapy Protocols↗

Proteinuria due to suboptimal hydration with high-dose methotrexate therapy.

One of the major complications after high-dose methotrexate (HDMTX) infusions is renal damage. We investigated the occurrence of proteinuria after HDMTX administration in children with pediatric malignancies (acute lymphoid leukaemia, osteosarcoma Burkitt's lymphoma). In the period 1989-1990 we gave 52 HDMTX courses to 24 children. During this period, prehydration and extra urinary alkalisation were performed only if the urinary specific gravity was over 1010 or if the urinary pH fell below 7. Using this schedule the mean values obtained for protein extraction were: before the therapy, 0.12 +/- 0.03 g/m2; on day 1 after MTX treatment, 0.38 +/- 0.06 g/m2; and on day 2 after the MTX infusion, 0.39 +/- 0.11 g/m2 (P < 0.01). A significant increase in proteinuria (> 0.2 g/m2 post- vs pretreatment) was detectable in 54% of the patients. In the period 1991-1992 we modified the hydration-alkalisation schedule to include i.v. prehydration for 18-24 h at 3 l/m2/day with a 0.45% NaCl-5% glucose solution along with sodium bicarbonate and posthydration for 72 h with the same solution. On this protocol the mean values determined for the urinary protein content were all in the normal range (pretreatment, 0.03 g/m2/day; day 1, 0.05 g/m2/day; and day 2, 0.08 g/m2/day). These findings were significantly different from the previous results (P < 0.05).

Adolescent↗

[Wernicke encephalopathy in childhood osteosarcoma].

Two children with osteosarcoma are presented in whom Wernicke encephalopathy with vomiting occurred during the chemotherapy. One of the children died with symptoms of toxic cardiomyopathy. Autopsy revealed Wernicke encephalopathy. The other child had similar symptoms (ocular signs, ataxia, somnolence). Parenteral thiamine had been given and after this therapy the child recovered from the encephalopathy. The authors emphasize the importance of the recognition of this neurological disorder occurring rarely in childhood: it can be cured with parenteral thiamine. Without thiamine treatment this condition is lethal.

Adolescent↗

[Experience with surgical treatment of osteosarcoma].

The authors treated 79 patients with osteosarcoma by surgery and chemotherapy 1975 through 1991. The appearance, localization and differential diagnosis are discussed. Attention is called to the doctor's delay. The effective pre- and postoperative chemotherapy decreased the incidence of the lung metastases and increased the survival rate (71% 3 years overall survival and 41% 5 years survival) and made the limb-sparing surgery possible. The development of the reconstructive surgical technique and new designs of tumor endoprostheses enabled us to perform limb-sparing surgery in more than one third of the cases at the same survival chance. The limb-sparing means a decisive change in the quality of the rehabilitation of the osteosarcoma patients.

Adolescent↗

Immunoglobulin prophylaxis during intensive treatment of acute lymphoblastic leukemia in children.

60 children with acute lymphoblastic leukemia were sequentially randomized at the time of diagnosis: Immunoglobulin (Endobulin, Immuno) was administered intravenously to 30 patients at a dose 100 mg/kg/week during the first 3 months, followed by 2 x 200 mg/kg/month immunoglobulin during the 4., 5., 6. months. No immunoglobulin was administered to the control patients. We studied the effect of immunoglobulin prophylaxis on the number of days with fever, number of cases with bacteriologically proved infections, length and frequency of antibiotic therapy. Our data confirm the efficacy of immunoglobulin prophylaxis during the intensive phase of leukemia therapy in children.

Child↗

Neuropsychologic and CT examinations in leukemic patients surviving 10 or more years.

Long-term survivors of childhood leukemia were studied to determine their neuropsychologic status. All had had cranial irradiation 10 or more years before. The results were 1. The IQ and academic performance of 56 cured leukemic patients show no significant difference from the normal population. 2. More exact neuropsychological tests (attention, reaction time, visual-motor coordination, memory) reveal important deficits. 3. The frequency of CT aberrations in 33 study patients was 45% and showed no correlation with either the IQ or other neuropsychological results.

Adolescent↗

[Neurologic tests in children having recovered from leukemia].

56 long-term survivors of childhood ALL have been investigated with CT and neuro-psychological methods. At the CT examination we found that 45.4% of the cured patients had alterations. Short-term memory, attention, visual-motor coordination and IQ were found to be slightly impaired in this group. Nevertheless they didn't influence the social integration and educational achievement of long-term survivors of ALL.

Adolescent↗