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Biomedical subjects

H Gadner

Publications and source records attributed to H Gadner.

At least 271 records · Page 15Linked to original sources
PubMed1987

[Aseptic bone necroses in leukemia patients in childhood and adolescence].

Four children with leukemia (1.6% of all leukemia patients) treated between 1979 and 1984 developed aseptic bone necroses, all of them at multiple sites. The average time from starting chemotherapy to developing bone necroses has been 19 months (range 9 to 28 months). This is a well known complication of corticoid therapy, but corticoids may not be the only aetiological factor. Other antineoplastic agents and leukemia for itself have been associated with aseptic bone necroses. Bone and joint pain caused by aseptic bone necroses can mimic leukemic relapse, so the diagnosis may be difficult. The increasing number of long term survivors in childhood leukemia, who underwent aggressive polychemotherapy, could make this problem more common in the near future.

Adolescent↗
PubMed1987

[Malignant peripheral neuroepithelial tumors in childhood].

The different therapy modalities and course of disease of 42 patients with a malignant peripheral neuroepithelial tumor are retrospectively analyzed. Therapy was completed in 31 children, 25 of whom had a primary localized tumor and 6 a disseminated neuroepithelioma. 17 of the children with a localized illness survive disease free in contrast to no survivor in the group with a disseminated tumor. The effective chemotherapy combining vincristine, adriamycin, ifosfamide and actinomycin D must be complemented by an efficacious local control because malignant peripheral neuroepithelioma tend to recur locally. The prospective analysis of newly diagnosed patients and a standardized therapy regimen will show if malignant peripheral neuroepithelioma represents a distinct tumor entity, different from a Ewings-Sarcoma.

Adolescent↗
PubMed1986

Cytogenetic studies in bone marrow transplant recipients.

Chromosome studies were performed in 24 patients who underwent allogeneic bone marrow transplantation (BMT) for severe aplastic anaemia (8), chronic myeloid leukemia (5 in chronic, 2 in accelerated phase and 1 in lymphoid blast crisis), acute myeloid leukemia (6), acute lymphoblastic leukemia in relapse (1) and Hodgkin's disease (1). Donor-cell type engraftment was demonstrated in 21 patients: in all 17 sex-mismatched transplants and - as demonstrated by reconstitution with Ph-negative cell populations - in 4 CML patients with a sex-matched donor. Recipient-type mitoses were seen in the bone marrow of 5 cases (1 SAA, 3 CML, 1 AML) after transplantation. They were only observed on one occasion in patients with SAA (4 of 25 on day 33) and AML (44 of 50 on day 14). Despite the continued demonstration of some Ph-positive mitoses in 3 patients with CML up to day 28, 323 and 451 after BMT, respectively, all surviving CML patients are still in complete haematological and clinical remission. So far the significance of these cytogenetically abnormal persisting host cells remains unknown.

Adolescent↗
PubMed1986

Toxicity of sequential high-dose ARA-C asparaginase treatment in childhood poor risk leukemia.

Seventeen children and two adolescents, aged 6 months to 20 9/12 years, with poor risk leukemia were treated with a total of 38 sequential high-dose ARA-C-Asparaginase courses (HIDAC-ASNase). Each course was followed by profound myelosuppression. Fever occurred in 13.2% and infectious complications in 7.9% of courses. Other side effects were vomiting (81.6%), drug fever (55.3%), mucositis and diarrhoea (28.9%), mild hepatotoxicity (26.3%), exanthemas (18.4%), conjunctivitis (15.8%), local ASNase hypersensitivity (7.9%), athropathy (5.3%). One patient developed generalized seizures followed by coma and death. The possible association between ARA-C, the CNS symptoms and death could neither be demonstrated nor excluded. Except for the possible ARA-C related CNS toxicity, toxic effects were reversible. We consider this treatment a tolerable chemotherapeutic contribution in childhood.

Adolescent↗
PubMed1986

Constitutional interstitial deletion of 11p11 and pericentric inversion of chromosome 9 in a patient with Wiedemann-Beckwith syndrome and hepatoblastoma.

A constitutional interstitial deletion on the short arm of chromosome #11 and an inversion of the heterochromatin of chromosome #9 were detected in a 1.5-year-old boy with Wiedemann-Beckwith syndrome (WBS) and hepatoblastoma. Of 37 malignant and nine benign neoplasms reported in approximately 250 cases with complete and incomplete forms of WBS, this is the fourth patient with hepatoblastoma. To date, 28 cases of WBS have been cytogenetically investigated with banding techniques. Constitutional anomalies have been found in only nine cases: Various anomalies resulting in a common triplication of the 11p15 region in six cases, reciprocal translocations t(11;22) and t(X;1) and an inversion of chromosome #2 in the three remaining cases. Triplication 11p15 was only present in one of four cases with a tumor. The breakpoints of the unique del(11)(p11.1p11.2) present in our case are proximal to those of del(11p13-11p14) and dup(11p15) observed thus far in both the aniridia-Wilms' tumor association and in WBS. Inversion of chromosome #9--one of the heterochromatin variants associated with elevated chromosomal instability, increased congenital abnormalities, and cancer proneness--may have been causally connected with a genetic imbalance resulting in the de novo deletion of 11p11. Therefore, we suggest that in these high-risk groups, C-banding studies should be performed together with high resolution chromosome analysis in order to also reveal the incidence and significance of C-band variants in individuals with such cancer prone syndromes.

Beckwith-Wiedemann Syndrome↗
PubMed1986

Treatment of severe aplastic anemia with combined immunosuppression (antithymocyte globulin and high-dose methylprednisolone).

Fifteen patients with transfusion-dependent severe aplastic anemia (SAA) were treated with combined immunosuppression consisting of horse-antithymocyte globulin (ATG; Atgam, Upjohn) and high-dose 6-methylprednisolone (MP). Oxymetholone was scheduled for 2 years but was discontinued in 7 patients after 10-385 days due to liver toxicity. Serious side effects usually seen in ATG monotherapy were rare during combined immunosuppression. Currently 12 of 15 patients are alive 110-1,275 days (median 475.5) after start of treatment. One patient has received too short treatment to be evaluated. All the others are transfusion-independent. Three patients died; two from septicemia before hemopoietic recovery could be expected and one after relapse. Our results confirm that the addition of high-dose MP abrogates the side effects of ATG monotherapy, and the addition of MP does not counteract, but rather enhances the beneficial effect of ATG in SAA. We recommend combined immunosuppressive treatment with ATG and high-dose MP as a highly feasible, safe and effectful therapy for patients with transfusion-dependent SAA.

Anemia, Aplastic↗
PubMed1986

[Treatment strategies in malignant non-Hodgkin lymphomas in childhood].

The prognosis of malignant non-Hodgkin lymphoma (NHL) in childhood has significantly improved due to stage-depending intensity and duration of treatment as well as the use of different therapeutic managements in non-B and B-lymphoma. According to the results of two consecutive BFM-studies (BFM-NHL 75/81, n = 116; BFM 81/83, n = 95) a curative rate of 70% may be reached with an acceptable therapeutic toxicity (under 2% deaths in the BFM-NHL-study 81/83). This result was already achieved in non-B-NHL in the study 75/81 by using an ALL therapy protocol. An improvement in the outcome of B-NHL was only achieved in the study 81/83 by introducing a new therapeutic concept including a combination of other agents and by reducing the duration of treatment to 8 weeks (stage I and II) and to 20 weeks (stage III and IV), respectively. In contrast to the non-B NHL, the prognosis of B-NHL distinctly varies according to the localized (I and II) and disseminated stage (III and IV). The probability of eventfree survival in the localized stage of B-NHL is excellent in both BFM-studies (86-100%). In the disseminated stage of B-NHL the result is not satisfying so far (61% in the study 81/83 vs. 38% in the study 75/81). The relapse cascade in non-B NHL proceeds similar to ALL in childhood. No relapses have been observed within 5 years after diagnosis. Recurrence in B-NHL only occurs within the first year after diagnosis which justifies the reduction of treatment duration.

Adolescent↗
PubMed1986

Aclacinomycin-A in the induction treatment of childhood AML.

In the cooperative study AML-IGCI-84 27 children with AML (FAB M1 7X, M2 4X, M3 1X, M4 6X and M5 8X; 1 megakaryocytic leukemia) have been treated. The median initial white blood cell count was 18.0 G/l (range 1.8-1,350.0 G/l). 1 or 2 courses of induction therapy were used: I1 (aclacinomycin-A (ACLA-A), VP-16 and ARA-C) and I2 (daunorubicin (DNR), VP-16, and ARA-C). I2 was used only if bone marrow contained greater than 5% blast cells on day 21. I2 and consolidation treatment were identical with the current AML-BFM-83 protocol. 3 deaths before day 21 occurred (2 cerebral hemorrhages, 1 septicemia). 24 patients were evaluable for response, 20 (83.3%) achieved CR, 16 (66.7%) by I1, 4 after I2. 4 patients never reached CR, 3 of them had a PR after I1. M5 patients did badly (2 early deaths, 2 PR, 4 CR). All patients without CR after I1 received the whole AML-BFM-83 protocol. Comparison of the results of the 2 studies revealed a similar CR rate for I1 (our patients) and I2 (BFM data): 80.0% vs. 82.2% (calculated for patients who ever reached CR). CR was reached before consolidation in all our CR patients compared to 82.2% of BFM patients. Early CR may be of long term prognostic significance. Cardiotoxicity of induction may be reduced by substitution of DNR by ACLA-A.

Aclarubicin↗
PubMed1986

Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen.

We report the case history of a 6 1/2-month-old girl with a hemophagocytic syndrome, pancytopenia, and excessive hepatosplenomegaly. Some extraordinary histological features present in this case--restricted organ involvement, excessive hemosiderosis, and fibrosis of the spleen--further contributed to the well-known problem of distinguishing between infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.

Diagnosis, Differential↗
PubMed1986

[Results of neuroblastoma treatment in Austria (1979-1984)].

During the period from 1979 until 1984 55 children were registered: 4 in Evan-stage I, 13 in stage II, 16 in stage III, 18 in stage IV and 4 in stage IV-S. 39 of these 55 patients have been followed for a minimum of two years. The current two year survival rate is 10/10 in stages I and II, 6/13 in stage III, 3/14 in stage IV and 2/2 in stage IV-S. These results demonstrate but a slight improvement in patients with stage II since the last Austrian statistic 1974. Despite modern chemotherapy the chance for survival of children with stage III or IV neuroblastoma is poor and better treatment modalities should be searched for.

Antineoplastic Combined Chemotherapy Protocols↗
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↗
PubMed1985

Surface glycoproteins (S-GP) on normal and malignant human leukocytes.

This study aimed to investigate high molecular weight surface glycoprotein (S-GP) patterns on various types of human leukocytes. S-GP were externally labelled by the Galactose-oxidase-NaB3H4 technique. Results based on the analysis of 120 samples derived from different types of normal and malignant leukocytes indicate that the relative expression of high molecular weight S-GPs changes during haemopoietic cell differentiation and to some extent these changes enable the classification of human leukocytes.

Antigens, Surface↗
PubMed1985

Hepatitis B vaccination and immune response in children with malignant diseases.

Fifty children with malignant diseases were vaccinated against hepatitis B. Twenty-nine children suffered from leukaemia or non-Hodgkin's lymphoma; 14 of these were on intensive chemotherapy (group I) and 15 were without intensive therapy (group II). The other 21 children had various forms of solid tumours, 14 of them were on intensive therapy (group III) and 7 were without intensive therapy (group IV). To evaluate the immune response, we determined antibody titres over a period of more than 14 weeks after the first vaccination. As 22 out of 50 patients had received passive immunisation together with either the first or the first and second vaccination, antibody titres at the 14th and 18th week (i.e. more than 10 weeks after passive immunisation) were used to evaluate the vaccination results. An antibody titre of greater than or equal to 10 mIU/ml was considered to be a positive response. All patients of group IV, but only 4 out of 14 in group III, 4 out of 15 in group II, and 0 out of 14 in group I produced antibody titres higher than 50 mIU/ml. In contrast to the full response in group IV, two-thirds of all other patients had no immune response (less than 10 mIU/ml). Based on our experience we recommend vaccinating patients suffering from solid tumours and receiving no intensive therapy (group IV) against hepatitis B and protecting all the other children with malignant diseases by passive immunisation, if necessary.

Adolescent↗
PubMed1985

Preliminary results of the GPO-trial for treatment of non-testicular germ-cell tumors in children and adolescents.

Germ cell tumors account for a 3% mortality rate of childhood malignancies. The combined chemotherapy, vinblastine, bleomycin and cis-platinum, is probably associated with more toxicity and treatment-induced mortality in children. Based on pharmacokinetic findings concerning the renal elimination of bleomycin and the well known cis-platinum nephrotoxicity, these two drugs were given sequentially. In addition, the cumulative dose of bleomycin was limited to 180 mg/m2. 61 patients entered the trial whereby 42 of these are evaluable. The chemotherapeutic regimen was well tolerated and was completed in 19 patients without treatment induced mortality and without evidence of pulmonary fibrosis. The treatment regimen seems to be successful despite the relatively low cumulative doses of vinblastine and bleomycin, as only 4 of 19 patients relapsed after a median observation period of 12 months.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

5q- chromosome in acute leukemia with lymphoid morphology and expression of myeloid membrane determinants.

We present three patients, two children and one adult, with an unusual type of acute leukemia. Whereas the blast cells showed lymphoid morphology with correlating cytochemical staining, immunological phenotyping exhibited a pure myeloid in one patient and a biphenotypic membrane marker profile in the other two patients. Cytogenetic studies revealed a 5q- chromosome as a common marker and additional individual changes. Two of the patients who were treated according to acute lymphoblastic leukemia (ALL) therapy protocols died without remission five and four weeks after diagnosis, respectively. Despite relapsing several times, another patient survived for over eight years. These three patients seem to represent one new subgroup of leukemias that can only be distinguished from typical ALL by both determination of cell surface markers and cytogenetic analysis.

Aged↗
PubMed1985

[Electrolyte changes in acute leukemia in childhood].

Electrolyte imbalance in leukemia can be due to either organ infiltration and cell death or to a side effect of cytostatic drugs. From the wide variety of these disturbances seen in acute leukemias in childhood, the excess of potassium is most dangerous. Further electrolyte changes, which are however less evident, are hyperphosphataemia, hyperphosphaturia, and hypocalcaemia. The destruction of a large amount of cells during aggressive induction therapy can boost the electrolyte imbalance and therefore lead to renal failure. Such situations are demonstrated in two cases. Following Vincristine and Cyclophosphamide administration, electrolyte changes such as acute or prolonged decrease of sodium in the serum and urinary loss of sodium are seen frequently. Based on the data from 20 patients with acute lymphoblastic leukemias we describe the dynamics of this process. These changes are probably caused by the syndrome of inadequate ADH-secretion. The clinical importance of these findings are discussed and procedures for improving therapy are set out.

Abdominal Neoplasms↗
PubMed1985

5q - marker chromosome in acute leukemia with lymphoid morphology and myeloid differentiation antigens.

We present three patients, two children and one adult, with an unusual type of acute leukemia. Whereas the blast cells showed lymphoid morphology with correlating cytochemical staining, immunological phenotyping showed a pure myeloid in one and a biphenotypic (mixed lymphoid/myeloid) membrane marker profile in two of the patients. Cytogenetic studies revealed a 5q - chromosome as a common marker with additional individual changes. Two of the patients who were treated according to ALL-therapy protocols died without remission 5 and 4 weeks after diagnosis, respectively. Despite relapsing several times, the third patient survived for over 8 years. These three patients seem to represent one new subgroup of leukemias which can only be distinguished from typical ALL by determination of both cell surface markers and cytogenetic analysis.

Aged↗