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Biomedical subjects

P Bucsky

Publications and source records attributed to P Bucsky.

51 records · Page 3Linked to original sources
PubMed1981

[Perineal neoplasms].

The literature on perineal neoplasms is reviewed and their incidence, the method and effectiveness of their surgical treatment analyzed. The prognosis of perineal neoplasms is tragic: according to published data 50% of the patients die within two, 92% within four years. The benign or malignant nature of perineal tumours can be judged only on the basis of histological tests. Attention is drawn to the necessity of the early surgical removal of perineal tumours. In the case of a 66-year old woman the histological diagnosis was gelatinous adenocarcinoma. To the best of the knowledge of the authors no similar case has been reported in the literature.

Aged↗
PubMed1979

[Isolation of immune complexes from the vascular wall in experimental immunovasculitis].

In experimental immunvasculitis immunocomplexes of the vessel wall have been detected by indirect immunofluorescence method. In the aortic intima linear deposition, in the small arteries and arterioles segmental fluorescence was observed in all layers of the vessel wall. In some regions a marked positive perivascular reaction was also revealed. Immuncomplexes of the vessel wall may play a part in the preservation and prolongation of the alterations of the vessel wall.

Animals↗
PubMed1978

The 1-hour D-xylose test in the diagnosis of villous atrophy.

The results obtained in 40 cases of intestinal mucosal biopsy and parallel 1-hour D-xylose test are presented. Of 40 patients, 15 had subtotal villous atrophy, while in 25 the villous structure was intact. Among the 15 patients with villous atrophy 10 gave D-xylose values of less than 20 mg per dl. In the control group all D-xylose values were above 20 mg per dl.

Biopsy↗
PubMedDate not supplied

Electron microscopic study of infantile renal biopsy specimens.

Out of 54 open renal biopsies performed on children, 17 were selected and studied not only by light microscopy but also by electron microscopy. Statistical-mathematical analysis of basement membrane thickness-measurements was carried out. It is concluded that electron microscopic investigation of the renal biopsy material is required in selected cases involving diagnostic or therapeutic problems. In nephrotic syndrome it may help above all to separate minimal changes from early forms or glomerular lesion of the focal sclerosing type. Electron microscopy can also be useful in any of the mono- and oligosymptomatic renal diseases. In those cases only electron microscopy makes it possible to provide exact morphological diagnosis which is necessary to the proper therapy.

Child↗
PubMedDate not supplied

Real-time polymerase chain reaction (RQ-PCR) for the monitoring of Epstein-Barr virus (EBV) load in peripheral blood mononuclear cells.

Measurement of Epstein-Barr virus (EBV) load is useful in peripheral blood for detecting primary and reactivated EBV-infections especially in immunosuppressed patients being at high risk for developing posttransplant lymphoproliferative disorder. For quantification of EBV DNA in peripheral blood of patients two real time polymerase chain reaction (RQ-PCR) assays were developed detecting sequences specific for the BAM HI-W and BAM HI-K region of EBV. In order to determine the optimal material of peripheral blood for RQ PCR analysis, DNA preparations of whole blood, peripheral blood mononuclear cells (PBMC) and B cells from 11 healthy, EBV-seropositive individuals were analysed in parallel and compared with regard to efficiency and sensitivity. While in whole blood preparations inhibitors of RQ PCR were detected influencing sensitivity, analysis of B cells being most sensitive is limited by being too labour intensive. In contrast, analysis of DNA preparations of PBMCs is sensitive enough to frequently detect EBV-specific sequences in all individuals tested and the preparation of PBMCs itself needs only a reasonable time. Thus, longitudinal monitoring of EBV load in peripheral blood of patients is possible by RQ-PCR, the optimal material for analysis being PBMCs.

B-Lymphocytes↗
PubMedDate not supplied

Immunodeficiency and Hodgkin's disease: treatment and outcome in the DAL HD78-90 and GPOH HD95 studies.

BACKGROUND: Excellent treatment results have been obtained for children with Hodgkin's disease (HD). Children with immunodeficiencies who present with HD do not have such a favourable prognosis. PATIENTS AND METHODS: A systematic literature search using MEDLINE and a search for immunodeficiencies in the database of the trials DAL HD78-HD90 and GPOH HD95 (n = 2263) were carried out. Age, sex, type of immunodeficiency, disease stage, treatment and outcome of all HD cases with known immunodeficiency were recorded. RESULTS: 28 published cases and 13 children in the DAL/GPOH trials were identified. 19/28 and 6/13 patients have immunodeficiencies with increased DNA breakage (24/25 ataxia teleangiectasia, 1/25 Nijmegen breakage syndrome) who present largely with stage III - IV HD. Among the published cases with increased DNA breakage there is only one child who is surviving 16 months after diagnosis, while there are 6/9 survivors in the group of immunodeficiencies without increased DNA breakage. Similarly, only 1/6 children survives in the group of children reported to the DAL/GPOH trials suffering from HD and immunodeficiency with increased DNA breakage, while the outcome in children suffering from immunodeficiency without increased DNA breakage is much better with 5/7 survivors. CONCLUSIONS: The literature review and data analysis of the DAL/GPOH studies show that treatment outcome is almost invariably fatal in children with HD and immunodeficiency with increased DNA breakage. Thus we propose to treat children with or without increased DNA breakage differently to improve the outcome of Hodgkin's disease in the subgroup of children with immunodeficiency.

Adolescent↗
PubMedDate not supplied

Failure to thrive in a 14-month-old boy with lymphopenia and eosinophilia.

We describe the case of a 14-month-old boy with delayed-onset SCID due to ADA-deficiency which was masqueraded only by failure to thrive. Remarkably, the child had no serious infections and an adequate immune response. However, absolute lymphopenia, eosinophilia and absent thymus on chest x-ray were indicative for immunodeficiency.

Adenosine Deaminase↗
PubMedDate not supplied

[Definition of malignant histiocytosis and large cell anaplastic (Ki-1) lymphoma in children].

Cells belonging to the mononuclear phagocytic system may give rise to a wide variety of proliferative disorders. These neoplastic or reactive diseases of monocytes, histiocytes and macrophages are still poorly understood, both from a biological, clinical and pathological point of view. In 1984 a new type of non-Hodgkin's lymphomas has been described, which recently was termed large cell anaplastic lymphoma (LCAL). Since both "true" malignant histiocytosis (MH) and LCAL display some clinico-pathological similarities, we reevaluated eleven children with the primary diagnosis of MH, who entered the multicentric therapy study DAL-HX 83. On the basis of the typical morphology LCAL and not MH has been diagnosed in all cases. The Ki-1 (CD 30) positive tumor cells of LCAL can display different phenotypes, either T-lymphoid, B-lymphoid, histiocytic or neither of them (O-phenotype). Therefore we investigated the lineage specificity of tumor cells in 15 paediatric patients with LCAL. Tumor cells of histiocytic origin could be demonstrated in only one of them. In 7 patients the neoplastic cells were of T cell and in the remaining 7 cases of non T/non B cell (O) phenotypes, confirming the extreme rarity of MH in children. It is our opinion that MH and LCAL seem to be different diseases not only nosologically but also clinico-pathologically. In the multicentric therapy studies NHL-BFM 81 and NHL-BFM 83 the frequency of LCAL in childhood is compared to all other subentities at 3%.

Adolescent↗
PubMedDate not supplied

[Large cell anaplastic lymphoma in children--clinical experiences with a newly defined histologic entity].

The clinical characteristics of 31 patients (pts.) (17 boys, 14 girls, median age 12 11/12 years) with large cell anaplastic lymphoma (LCAL) have been evaluated. 17 of these pts. had originally been diagnosed as suffering from "malignant histiocytosis" ("MH") and were therefore included in the DAL-Histiocytosis X 83 study. Another 14 pts. with Ki-1 lymphomas were enrolled in the BFM-NHL therapy studies. According to Murphyclassification 24 pts. (77%) had stage III or IV disease and in general presented in a severe condition. The lymphatic system was involved in 28 pts., 8 pts. (26%) had skin infiltration. With regard to lymphoma involvement of lung, bones and bone-marrow were unexpectedly frequent. CNS involvement was seen in just one pt. Despite rather heterogeneous therapy approaches (ALL-schedules, DAL-HX 83 protocol for treatment of "MH", combination of B-NHL-BFM and AML-BFM schedules, CHOP, BFM protocols for B-NHL) 30 out of 31 pts. achieved clinical remission (CR). The only nonresponder died during bone marrow transplantation of septicemia. 4 pts. relapsed during therapy. 3 of them died, 1 during a BMT. 1 pt. achieved 2nd CR with a BFM-B-NHL protocol. 3 pts. experienced a late relapse, 1 died, 1 2nd CR was achieved, the third pt. is still alive after 2 further relapses disease-free for 3 years. 23 pts. (74%, 13 out of 14 of BFM-NHL therapy study, 10 out of 17 of DAL-HX 83 study, 1 pt. after BMT) are in 1st CR with a median observation time of 2 9/12 years (range 5/12 to 17 9/12 years).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

Low grade malignant non-Hodgkin's lymphomas and peripheral pleomorphic T-cell lymphomas in childhood--a BFM study group report.

Non-Hodgkin's lymphomas (NHL) are classified as low or high grade malignant lymphomas of either B or T cell origin. Cells of low grade malignant NHL (LG-NHL) of B cell type represent either follicular or postfollicular, cells of LG-NHL of T cell type postthymic maturation stages in the lymphoid differentiation pathway. LG-NHL often fail to undergo long-term complete remission or cure in adults, no matter what type of conventional therapy is applied. In three pediatric therapy studies frequency and probability of continuous complete remission (pCCR) were studied for LG-NHL and peripheral pleomorphic T-cell lymphomas (PTCL). Of 432 evaluated patients only six children (1.4%) were qualified as LG-NHL. LG-NHL of T cell type were diagnosed in three children (one T-zone lymphoma, two PTCL of small cell type), LG-NHL of B cell type in another three cases. Two children presented with nodular type of centroblastic/centrocytic lymphoma (CB/CC), one with lymphoplasmocytoid type of immunocytoma (IC). In addition to the two patients with low grade malignant PTCL, there have been also four children with high grade malignant PTCL. The analysis showed that there was not significant difference for event free survival of children with LG-NHL and HG-NHL, respectively (pCCR: 0.63 and 0.82, p = 0.29). In contrast, when comparing high and low grade PTCL versus all other types of childhood NHL (non-PTCL), a significant difference seems to exist (pCCR for PTCL: 0.44, for non-PTCL: 0.83; p = 0.02). However, for further concisive conclusions more patients are needed for evaluation.

Adolescent↗
PubMedDate not supplied

[Acute lymphoblastic leukemia in infancy: results of 5 multicenter ALL-BFM therapy studies 1970-1986].

Acute lymphoblastic leukemia (ALL) of infancy has in contrast to all other age groups a less favorable prognosis. In order to determine the possible causes and biological principles for impaired outcome of infant patients in five consecutive clinical trials ALL-BFM 1970-1986, both clinical characteristics and biological features for one hundred and ninety-six patients aged two years and less have been evaluated retrospectively (forty-two infants under one year of age). The observations illustrate, that less favorable subtypes of childhood ALL are more frequent in these children, preferably in infants aged six months and less, explaining decisively the impaired prognosis. These subtypes are characterized by large tumor burden (p = less than 0.001), initial central nervous system (CNS) involvement (p = less than 0.001), and a high CNS relapse rate (p = 0.03). Phenotypically the undifferentiated leukemic blast cells show negative reactions for cALLa and often for Tdt (0-ALL and AUL; p = less than 0.001). The switch to prognostically more favorable ALL subtypes occurs about the end of the first year of life. Nevertheless, early failures by non-response or late-response seem not to influence the poorer outcome. However, compared to the preceding trials, results of studies ALL-BFM 81 and ALL-BFM 83 were significantly improved in respect to the probability of continuous complete remission for infant patients (pCCR less than 1 y: 0.44 vs. 0.28), confirming that treatment by itself is one of the major prognostic determinants.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Cellular drug resistance in acute myeloid leukemia: literature review and preliminary analysis of an ongoing collaborative study.

Cellular drug resistance is one of the main causes of the frequent ultimate failure of chemotherapy in childhood acute myeloid leukemia (AML). We here summarize the results of a literature review on in vitro drug resistance in childhood AML, focusing on studies using so-called cell culture assays. We also briefly describe some results of an ongoing collaborative study between the Research Laboratory of Pediatric Oncology in Amsterdam (University Hospital Vrije Universiteit) and the German BFM-AML Group. In general, the literature and our preliminary data on in vitro cellular drug resistance in AML are promising in terms of clinical relevance. Cell biological features and clinical response to chemotherapy are related to in vitro drug resistance. However, a large study including multivariate analysis is required to more firmly establish the clinical value of cellular drug resistance testing in childhood AML, and the collaborative study will therefore be continued. Possible applications of cell culture assays include risk-group stratification, rational improvements of current treatment protocols for subgroups of patients based on specific drug resistance profiles, individualised tailored therapy, the study of cross-resistance patterns between drugs, the study of possibilities to modulate or circumvent drug resistance, the study of drug interactions, selection of patients for clinical phase II studies and drug screening.

Acute Disease↗
PubMedDate not supplied

Malignant endocrine tumours in childhood and adolescence--results of a retrospective analysis.

BACKGROUND: Malignant endocrine tumours (MET) are rare neoplasms in childhood and data on such tumours are scarcely available. The aim of this retrospective study was to collect data over a period of 11 years (1984-1995) and to give the basis for a prospective study. PATIENTS AND METHOD: 180 departments of paediatrics, nuclear medicine and children's surgery were asked for reporting of patients with MET by a questionnaire. 35% of the departments answered (however, 75% of the paediatric departments). RESULTS: 96 children with MET were reported: 73 with thyroid cancer (50 papillary, 15 follicular, 7 medullar, 1 anaplastic; 1:1,9 boys to girls, both mean and median age 11 3/4 years), 12 with adrenocortical cancer and 11 with other malignant endocrine tumours. Metastases were found at diagnosis in 41 of 65 children with papillary or follicular carcinoma. 37 patients (pts.) are in first continuous complete remission (CCR), 4 in partial remission (PR) and in 24 (!) children the remission state or the outcome is not known. 6 of 7 children with medullary cancer have had metastases at diagnosis. 1 patient is in CCR, 1 patient is living in PR, 1 in relapse. 4 children are lost of follow up (lfu.). In sex-ratio no difference was found in 12 pts. suffering from adrenocortical cancer (mean age 5 1/4 years, median age 2 1/2 years). 11 tumours showed hormonal activity. 5 children disclosed metastases at diagnosis. All patients were treated by surgery, 6 received chemotherapy additionally. 4 children are living in CCR, 3 pts. in remission of unknown state, 4 died (all of them with metastases at diagnosis), 1 patient is lfu. The other MET reported: 8 carcinoids (7 x appendix, 1 x lung), 2 phaeochromocytomas, 1 islet cell cancer. 8 pts. are in CCR, 2 are lfu. The child with the islet cell carcinoma died. CONCLUSIONS: Since only 35% of the clinics answered this retrospective analysis cannot give any statement about the incidence of MET in children. As to the prognosis thyroid cancer seems to have a favourable prognosis in childhood and adolescence. In contrast, metastatic adrenocortical cancer is incurable in this age group. Carcinoids of the appendix can be treated curatively by appendectomy. To better understand the biology of MET in children and adolescence and to achieve a better prognosis for some types of these tumours, much more data are needed. For these reason a multicenter prospective therapy study for childhood MET seems to be necessary.

Adenocarcinoma, Follicular↗