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Biomedical subjects

P Krepler

Publications and source records attributed to P Krepler.

At least 37 records · Page 2Linked to original sources
PubMed1985

[Cooperative studies in the treatment of acute lymphoblastic leukemia in children in Austria--report of 10 years' experience].

437 children with acute lymphoblastic leukaemia (ALL) have been treated at 9 different institutions in Austria utilizing common protocols and central registration between 1974 and 1984. 227 patients (132 boys and 95 girls, group I) were treated between 1974 and 1980 using 3 consecutive protocols (KMK, O 76, A 78), which were essentially derived from the Memphis studies VII and VIII. Patients with a high risk of relapse were treated according to the LSA 2-L2 protocol. 210 patients (112 boys and 98 girls, group II) were consecutively treated following the BFM protocols 76/79 and 81/83. In this group, treatment intensity was adjusted to the initially determined individual risk of relapse (BFM risk score or risk factor). To date, the life table analysis demonstrates that the probability of continuous complete remission for patients in group II is 60% after 5 and 3 years (BFM 76/79 and BFM 81/83, respectively), whereas group I reaches a level of 37.3%. The prognostic difference between risk and non-risk patients in both studies of group II was eliminated. Despite a higher morbidity and non-leukaemia-related mortality in group II, the therapeutic success can be attributed to the intensification of induction therapy.

Asparaginase↗
PubMed1982

Results of LSA2-L2 therapy in 26 children with non-Hodgkin's lymphoma.

Twenty-six children with non-Hodgkin's lymphoma (NHL), 17 boys and nine girls, were treated with the LSA2-L2 protocol. Seven children had stage I or II, 16 Stage III and three Stage IV according to Murphy's staging system. Eight children had their primaries in peripheral lymph nodes, eight in the abdomen, six in the mediastinum and four in other sites. All tumors were classified histologically according to four different classifications. Overall disease-free actuarial survival is 53.6%. Complete responders show a disease-free survival of 77.8%. Fourteen children survived for 9-56 months. Included are all seven children with Stage I or II who survive irrespective of histologic type of the tumor. Of the remaining 12 children in Stage III or IV three children died in remission and nine of progressive disease. Eight of these nine patients did not attain complete remission. Whereas four of five children with the convoluted type of NHL survive, four of five patients with the Burkitt's type (small noncleaved follicle center cell lymphomas) died of progressive disease. According to Rappaport's classification, four of six children with diffuse undifferentiated lymphoma (DUL) are dead due to tumour progression. Considering the classification of lymphoblastic lymphomas introduced by Nathwani et al., 23 five of seven children suffering from lymphoblastic lymphomas but only two of eight children with nonlymphoblastic lymphomas belong to the survivors. Therefore histologic findings do hold prognostic significance in our series of children with NHL.

Adolescent↗
PubMed1982

[Progress in the treatment of juvenile leukemias].

Between January 1979 and December 1980 64 children with acute lymphoblastic leukemia were treated in 9 pediatric clinics in austria according to the BFM study 76/790-protocol. For remission induction all patients received an 8 week multidrug regimen (West-Berlin ALL-protocol). High risk patients were defined according to a risk score at diagnosis and additionally treated with a 6 week reinforced reinduction protocol during the first half year after diagnosis. Maintenance therapy was stopped after about 22 months. The life table-analysis after 30 months showed a 75.5% disease free survival for the total group of patients. Compared with a control group of 228 patients treated between 1974 and 1980 in 9 different clinics in Austria according to 3 consecutive national treatment regimens (modifications of Memphis protocol VII and VIII), therapeutic results were markedly improved. After a follow-up of 36 to 90 months the overall oumulative remission rate was 37.7%. The results could be improved especially in the group of high risk patients for replase by 35% in contrast to the historical studies. A prognostic difference between low- and high risk-patients was not seen in the BFM study (84.3% vs. 69.9%). Without doubt, the marked improvement of prognosis is due to the intensification of therapy.

Antineoplastic Agents↗
PubMed1981

[Treatment of Wilms' tumor (author's transl)].

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.

Age Factors↗
PubMed1981

[Effect of BCG vaccination of the newborn infant on the incidence and course of juvenile leukemias].

As a retrospective study the clinical data of 613 leukaemic children were collected. 269 of them were born in Austria, fell sick between 1967 and 1976 and were not older than 5 years at the onset of disease. BCG-vaccination, which is performed in Austria within the first three days after birth, was checked by means of hospital reports, birth protocols and central BCG record. All data were stored and evaluated by electronic data processing. Besides a significant decrease of mortality, which we could demonstrate in earlier studies, the following correlations between BCG-vaccination of newborn and leukaemia were found: Reduction of morbidity, decrease of case fatality, increase of 5 years survival rate, increase of mean survival time, delayed onset of disease, decrease of frequency of myeloic and chronic leukaemia. With the exception of the slight reduction of morbidity all correlations are significant.

BCG Vaccine↗
PubMed1980

[Unilateral congenital anophthalmos with Trabecular agenesia and hydrophthalmos of the other eye (author's transl)].

In a one-year-old female patient, unilateral anophthalmos was ascertained by clinical, echographic and X-ray examination. In addition, the cranial computer tomogram showed trabecular agenesia. The globe of the other eye was existent and was enlarged by hydrophthalmos. Pediatrically this case represents a syndrome with multiple anomalies of etiologically unknown origin; apart from the ocular malformation there are a severe cerebral developmental disturbance, an apallic syndrome, hypsarrhythmia, peculiar disproportional bodily structure with skeletal anomalies as well as an abnormal susceptibility to infections of the respiratory system and a marked, isolated IgA-deficiency. Neither chromosomal anomaly nor familiarity was discovered.

Anophthalmos↗
PubMed1980

Characteristics of a cell line established from a child with familial acute myeloid leukaemia.

The properties of an unusual new cell line from a child with acute myeloid leukaemia (AML) is described. The patient, his mother and grandmother all died within 2 years of AML. Karyotypic analysis suggests that the cells which grow in vitro represent a proliferation of the child's leukaemic cells. The presence of myeloid alkaline phosphatase in these cells suggests that they represent a proliferation of malignant myeloblasts. During the first year in culture a significant percentage of the cells formed E-rosettes, but the cells failed to react with monoclonal anti-T-cell antibodies. The cells are SmIg, Fc, C3 and EBNA negative. Ultrastructural examination of the cells revealed cytoplasmic inclusion bodies which contained virus-like particles. These inclusion bodies and particles were similar to those observed by us in two other human leukaemic cell lines and not unlike those in fresh human leukaemic cells, as reported by others.

Alkaline Phosphatase↗
PubMed1980

Con A-induced suppressor cells in children with acute lymphoblastic leukaemia.

The function of Con A stimulation was investigated in patients with ALL in remission and in patients with ALL in the acute phase of the disease as compared to healthy controls. Suppression of the Con A response brought about by autologous Con A-activated cells was significantly lower in ALL patients in remission (mean value 29.33%) and in the acute phase (mean value 10%) than in the controls (mean value 64.86%). Suppression of the Con A stimulation of control lymphocytes by Con A-activated homologous cells of ALL patients had a mean value of 51.65%. This was significantly higher than the suppression obtained by the same ALL cells in the autologous system and of the same order of magnitude as the suppression obtained by control Con A-activated cells on control Con A stimulation (57.67%). Suppression of the Con a response of ALL lymphocytes produced by control Con A-activated cells was 23.17% and comparable to healthy controls. These results demonstrate that the function of Con A-induced suppression is significantly lower in ALL patients. They further indicate that at least two cell types are involved in this kind of suppression in humans.

Acute Disease↗
PubMed1979

[Infections in children with malignant disease (author's transl)].

Infections of children with malignant disease, especially of the lympho-reticular system, are characterized by their severity, with a high mortality, as a consequence of defective immunocompetence. According to the immunosurveillance theory, temporary immune defects could have even facilitated the malignant growth. The neoplastic disease itself contributes to the immunodeficiency by multiple mechanisms. The powerful cytostatic-cytocidal drugs reduce the immune response also, especially in the phases of bone marrow depression. Granulocytopenia shows the most significant correlation with the incidence of serious infections. The different forms of hospital infections have been reviewed and classified as 1. bacterial, fungal and, rarely, (but most dangerous) protozoal infections, 2. endogenous infections with the patient's own anaerobic intestinal flora and 3. viral infections. The perspectives of up-to-date chemotherapy and management of the immunodeficiency e.g. with leucocyte transfusions, and attempts to prevent infection are discussed.

Amphotericin B↗
PubMed1978

[BCG vaccination and leukemia. Epidemiologic studies on the effect of BCG vaccination on leukemia].

The possible influence of BCG vaccination on leukemia mortality of infants was investigated by various authors. The methods used were prospective trials, retrospective studies and epidemiological analyses. In some of these studies a protective effect of BCG vaccination was observed, in other studies no influence was found. Up to now this problem is subject to controversial discussions. An epidemiological analysis of the Austrian data using trend analyses, regression analyses and analyses of rank correlation showed an inverse correlation between BCG vaccination rate of newborns and leukemia mortality in the age group 0--5 years. From this correlation a protection rate of 0.77 could be calculated.

Austria↗
PubMed1978

[The effect of neonatal BCG vaccination on the incidence of leukemia (author's transl)].

Data from several publications were analyzed with respect to the possible non-specific influence of BCG vaccinations on leukemia. Our own investigations in the five largest provinces of Austria showed that there was an inverse correlation between leukemia mortality and BCG vaccination of the newborn. From this data a protection rate of 0.88 for prevention of leukemia deaths could be calculated for the first five years of life. The number of positive tuberculin reactions was significantly lower in leukemic infants than in normal ones. This finding was completely in accordance with the probability of positive tuberculin reactions as calculated from the protection rate.

Austria↗
PubMed1978

[The prognostic value of lymphocyte transformation by phytohemagglutinin in children with acute lymphatic leukemia].

The response to phytohemagglutinin (PHA) of lymphocytes from 62 children with acute lymphoblastic leukemia (ALL) was tested before and during cytostatic therapy. Nineteen patients were tested as well for reactivity in autologous mixed lymphocyte-blast cell cultures. The prognostic value of the results obtained has been assessed. Children remained longer in their first complete remission 1) if the response to PHA before therapy was normal or only slightly diminished, or 2) if it returned to normal as soon as complete remission was attained, and 3) if periodic intensification therapy was followed by a rebound in spontaneous lymphocyte proliferation. The stimulatory response to PHA during maintenance therapy and the reactivity in mixed lymphocyte- blast cell cultures were not helpful prognostic criteria. In conclusion, determination of the PHA responsiveness at specific times, i.e. before therapy, after remission induction and after periodic intensification therapy, could help identify poor risk patients in ALL.

Antineoplastic Agents↗
PubMed1978

Immunological properties of lymphoblasts and lymphocytes in children with acute lymphatic or undifferentiated leukemia.

In 35 children with acute lymphatic or undifferentiated leukemia, immunological properties of normal lymphocytes and lymphoblasts were investigated. In most cases the number of circulating normal lymphocytes with surface or B markers and delayed type hypersensitivity was depressed. On the other hand, the response to mitogenic stimulation with PHA as well as the antibody and immunoglobulin serum concentrations were found to be normal. Lymphoblasts did not exhibit surface receptors in any but four patients, who showed 10--18% of leukemic blasts being positive for E-rosette formation at 0 degree but not at 37 degrees.

Acute Disease↗
PubMed1978

Preventive cranial irradiation in the treatment of acute lymphocytic leukemia in children.

From April 1971 to September 1977, 94 children with acute lymphocytic leukemia, in whom a complete clinical and hematologic remission had been obtained received preventive cranial irradiation at the University Clinic for Radiotherapy of Vienna. So far, only in 2 of the 94 patients an initial meningeal relapse occurred. After a minimum follow-up time of 3 years, the rate of primary CNS relapse is 8%. The combination of cranial irradiation and methotrexate intrathecally, beginning currently in the first month of the complete remission, seems to be the most effective and least hazardous regimen to prevent meningeal and CNS leukemia.

Adolescent↗