Successful treatment of orbital rhabdomyosarcoma in two infants using chemotherapy alone.
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Antineoplastic Combined Chemotherapy Protocols↗
Biomedical subjects
H Lackner
Publications and source records attributed to H Lackner.
Feasibility of peripheral blood stem cell (PBSC) and peripheral blood mononuclear cell (PBMNC) separation in children with a body weight below 20 KG.
Nine children from 10 to 76 months (median 28.0), weighing 8.5 to 19.7 kg (median 13.0 kg) underwent peripheral blood stem cell separation (PBSCS) or peripheral blood mononuclear cell separation (PBMNCS), after insertion of a double-lumen central venous catheter (8-10 French). Separations were performed with a continuous flow blood separator (Fen-wall CS 3000 plus), running a specially adopted separation-program. In 7 children (5 with neuroblastoma IV, 1 with multifocal Ewing's sarcoma, and 1 with rhabdomyosarcoma IV), stem cells were mobilized by application of G-CSF at a dosage of 15-27.7 micrograms/kg body weight (median 16.25) subcutaneously following high-dose chemotherapy, according to the disease-related protocols, whereas 2 children had PBMNCS to induce graft vs. leukemia (GvL)-reaction in the HLA-identical sibling suffering from relapsed chronic myelogenous leukemia (CML: n = 1), or chronic myelomonocytic leukemia (CMML: n = 1) after allogeneic BMT. In all cases, the collecting procedure was performed after filling the cell separator with priming solution consisting of 2 U of irradiated and washed packed red cells, 250 ml human albumin, and 0.9% NaCl. In the 7 patients with solid tumors between 0.45 and 62.7 x 10(6) CD-34 positive cells/kg body weight were separated; the patient who had the lowest yield was separated twice after another mobilizing course. Three patients (2 with neuroblastoma IV and 1 with multifocal Ewing's-sarcoma) underwent a double transplantation with 1-3 portions of the collected stem cells within a 5- to 6-week interval. Two children had a rapid engraftment on both peripheral blood stem cell transplantations (PBSCTs). The third child, who had the lowest yield and was separated twice had prompt engraftment at the first PBSCT but delayed and incomplete engraftment at the second PBSCT. One patient after adoptive immunotransfer with PBMNCs for relapsed CML is now 40 months in complete cytogenetic and molecular biological remission, whereas the other patient treated for relapsed CMML did not respond to the PBMNC-transfusion. The results indicate that PBSCS and PBMNCS can be performed in children with a body weight below 20 kg.
Antigens, CD34↗
[Eccrine hidradenitis. Case report and review of the literature].
Neutrophilic eccrine hidradenitis is a self-limited dermatosis with spontaneous resolution. The clinical presentation and location of the lesions are variable. Histopathologically, neutrophilic eccrine hidradenitis is characterized by a predominantly neutrophilic or mononuclear infiltrate around the eccrine ducts with associated necrosis. Possible causes include malignant hematological disorders, tumors, side effects of chemotherapy and bacteria infections. We report a 16-month-old female patient with idiopathic neutropenia undergoing G-CSF therapy, who suddenly developed numerous papules on her trunk and extremities. The lesions resolved spontaneously within 6 weeks without treatment. The clinical and histopathological findings of the hitherto published 45 cases are reviewed.
Biopsy↗
Connatal localized neuroblastoma. The case to delay treatment.
BACKGROUND: Spontaneous regression is well documented for a subset of widespread neuroblastomas (Stage 4S) and for localized residual tumors after incomplete resection. Possible spontaneous regression of untreated localized neuroblastoma in infants is frequently discussed, but has very rarely been demonstrated clinically. METHODS: We report four patients with localized neuroblastomas detected early; all were tumors of the adrenal gland. One patient was detected antenatally by ultrasound, the other three tumors were detected incidentally by sonography at age of birth, 1 week, and 7 weeks, respectively. In three patients treatment was delayed in order to await a possible spontaneous regression, and in one patient treatment was delayed due to an uncertain diagnosis. RESULTS: Local tumor growth was observed in three patients, and the tumors were removed 7, 12, and 16 weeks, respectively, after the initial diagnosis of the neuroblastoma. All three patients are free of disease. The fourth patient developed liver metastases 4 weeks after the first suspicion of neuroblastoma. Progressive disease ended in death at the age of 17 months. CONCLUSIONS: None of the four patients showed spontaneous tumor regression. Noninvasive examinations and invasive investigations (in two patients) were unable to predict the tumor's behavior. Based on present knowledge, a general "wait and see" strategy cannot be recommended for early and incidentally detected neuroblastoma patients.
Adrenal Gland Neoplasms↗
Screening for neuroblastoma in late infancy by use of EIA (enzyme-linked immunoassay) method: 115000 screened infants in Austria.
The aim of this study was to investigate the feasibility of a neuroblastoma screening programme for children in late infancy, based on collaboration of general paediatricians and practitioners in Austria, using the technique of enzyme-linked immunoassay (EIA) for biochemical analyses. Analysis of catecholamine metabolites in spot urine samples by EIA with high performance liquid chromatography as a backup was undertaken. Austrian infants (median age 8.7 months) were screened. Overall compliance was 30%. The EIA method had a high rate (6.7%) of false-positive results. 28 infants were admitted to hospital. In 15 cases, neuroblastoma was found (four stage 1, five stage 2B, six stage 3). The EIA method can be used for neuroblastoma screening, but requires a backup analytical technique in order to avoid unnecessary hospital admissions. The stage distribution and biological features of neuroblastomas diagnosed by screening at a later age are different from those detected by earlier screening. Screening in late infancy might be of more benefit than early screening.
Austria↗
[5 years neuroblastoma screening in Austria: rate of participation, results and a comparison with other screening areas].
In late 1990 a screening program for the early detection of neuroblastoma in infants was introduced in Austria. The program is performed on a voluntary basis in collaboration with general pediatricians and practitioners. Filter strips for urine collection are distributed to parents of infants aged seven to nine months on the occasion of a routine check up. The samples are sent to the laboratory by parents and analysed for vanillylmandelic acid (VMA) and homovanillic acid (HVA). Between January 1991 and December 1995 125,201 infants were screened. The compliance rate was 26.8% for Austria, but great differences were seen for different regions (65% in Carinthia, 10% in Vorarlberg). 30 children were admitted to hospital for investigation of repeatedly elevated urine catecholamines. A neuroblastoma was identified in 16 cases. In 12 of these cases at least one unfavorable prognostic factor was present (stage > or = 3, elevated LDH, unfavorable histology, N-myc amplification, di- or tetraploidy). Neuroblastoma screening of infants aged more than six months seems to detect predominantly those tumors which are unlikely to regress spontaneously. The observation of one false negative case, however, demonstrates that neuroblastomas which become clinically manifest at a later date may remain undetected by early screening. Possible advantages of shifting screening to a later age and repeated screening are discussed.
Austria↗
Ocular sequelae of multimodal therapy of hematologic malignancies in children.
We retrospectively analyzed the ocular findings after polychemotherapy including intrathecal methotrexate, systemic corticosteroids, and prophylactic cranial irradiation in children with acute lymphoblastic leukemia (n = 16) and non-Hodgkin's lymphoma (n = 2). After a median surveillance time of 4.1 years, asymptomatic ocular abnormalities were observed in 83% of the patients: 7/18 had a decreased tear formation, 5/17 had an opacity of the vitreous body, and 13/18 had an opacity of the lens. It was not possible to determine retrospectively which therapy caused a particular effect. A comparison of the 2 irradiation techniques (with and without blocking of the lacrimal glands) showed that in 5/7 children who developed a reduced eye secretion, the lacrimal glands are within the treatment volume. Therefore, the reduced eye secretion is most likely radiation-induced. Whereas the opacities of the vitreous body were caused by thrombopenia and bleeding during the course of disease, corticoid therapy might have contributed to the lens opacities.
Adolescent↗
Treatment of aplastic anemia with a monoclonal antibody directed against the interleukin-2 receptor.
Severe aplastic anemia (SAA) has been related in most cases to underlying autoimmune conditions. Various immunosuppressive regimens have been recommended in the absence of an HLA-identical bone marrow donor. Prednisolone, antithymocyte globulin, and cyclosporin A have been shown to be effective. This report describes the successful treatment of a 23-year-old woman suffering from severe aplastic anemia who had become multiresistant against previously administered immunosuppressive agents, using a monoclonal IL-2-receptor blocking antibody. The patient responded within 4 weeks. The time to the next relapse was 8 months; however, another remission with a second course of horse-antithymocyte globulin was achieved and has been maintained for 27 months to date with low doses of cyclosporin A. Although this is an anecdotical report, IL-2-receptor blockade using a monoclonal antibody might be considered as a further alternative in multi-resistant SAA, perhaps increasing the susceptibility to further immunosuppressive trials.
Adult↗
Liposomal amphotericin-B (AmBisome) for treatment of cutaneous widespread candidosis in an infant with methylmalonic acidaemia.
In a 10-week-old infant with vitamin B12-unresponsive methylmalonic acidaemia, cutaneous candidosis (Candida albicans) progressed rapidly despite topical antifungal treatment. After 1 week of intravenous therapy with liposomal amphotericin-B (AmBisome) the dermatitis disappeared completely and blood cultures were sterile. No side-effects were observed. This is one of the first experiences in the treatment of infants with this new antifungal agent.
Amino Acid Metabolism, Inborn Errors↗
The role of sonography in the evaluation of gastro-oesophageal reflux--correlation to pH-metry.
Sonography was compared to pH-metry and/or oesophagomanometry to evaluate the accuracy of sonography in the early diagnosis of gastro-oesophageal reflux. Thirty children with a mean age of 72 days (21-252 days) were studied. The results showed that specificity of sonographic diagnosis was 87.5% and sensitivity was 100% (with P less than 0.001). Sonography also proved helpful in providing both functional and morphological data in addition to pH-metric results. This study therefore suggests that sonography is useful as the first approach in the diagnosis of vomiting babies as it is non-invasive and provides sufficient diagnostic accuracy.
Female↗
Multiple emboli from a large aortic arch thrombus in a patient with thrombotic diathesis.
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Adult↗
[The effect of high-dose peroral zidovudine treatment in a 4-year-old child with HIV encephalopathy].
We report a four-year-old boy with HIV encephalopathy after vertical HIV infection. On the first admission to our hospital he showed ataxia, loose of expressive language and interstitial pneumonia. After treatment of the pneumonia the patient was started on oral zidovudine with 6 x 6 mg/kg bw/day, because of persisting neurologic symptoms and deep white matter lesions in the MR tomogram of the brain. Two months later he showed an improvement of the gait and the reappearance of the expressive language. Seven months after the start with zidovudine the MR tomogram of the brain revealed the disappearance of white matter lesions with exception of little areas of demyelinisation. No side effects of treatment were observed. The only persisting pathological clinical signs were developmental delay of about a half year and moderately hyperactive tendon reflexes of the lower extremities. Our case suggests that even oral treatment with zidovudine can have a beneficial effect on the HIV encephalopathy in infants.
AIDS Dementia Complex↗
Liposomal amphotericin-B (AmBisome) for treatment of disseminated fungal infections in two infants of very low birth weight.
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Amphotericin B↗
[Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].
Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation.
Adolescent↗
[You make the diagnosis. Lymphedema praecox--primary lymphedema].
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Adolescent↗
[Infections in the neutropenic phase following bone marrow transplantation: comparison of laminar airflow isolation with conventional isolation].
From May 1978 to September 1989 45 patients underwent 25 allogeneic and 21 autologous bone marrow transplantations (BMT) and 1 peripheral stem cell transplantation for the following indications: severe aplastic anemia (n = 4), hematological malignancies (n = 28), malignant solid tumours (n = 12) and sideroblastic anemia (n = 1). The first group of 20 patients was isolated in a conventional hospital room, while management of the aplastic phase in the second group of 25 patients was performed in a laminar air flow (LAF) unit. All patients received total decontamination. In a retrospective analysis the number of positive blood cultures during the neutropenic period was 85% in the first group, as compared with 40% in the second group, and the number of febrile episodes was 85% versus 64%, respectively. Despite the fact that the septic morbidity was lower in the LAF group, mortality during the neutropenic period (15% in group I versus 16% in group II) was unaffected and survival rate (45% in group I versus 36% in group II) did not improve. We conclude that LAF protection will only have a positive impact on survival rate if the incidence of non-infectious complications of BMT, such as organ toxicity or graft-versus-host disease, is likewise reduced.
Adolescent↗
Rapid and simple high-performance liquid chromatographic assay for 5'-fluorouracil in plasma for bioavailability studies.
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Biological Availability↗
Imipenem-antibiotic monotherapy in juvenile cancer patients with neutropenia.
From 1984 to 1987 two consecutive groups of juvenile cancer patients (n = 45) with fever and neutropenia corresponding in all criteria were examined. In half of the total of 90 febrile episodes and septicemias, a conventional antibiotic combination therapy (Pseudomonas-active penicillin/cephalosporin of the third generation/aminoglycoside) was instituted. In the remaining half imipenem was used as an antibiotic monoagent. In 66% and 60% of the febrile episodes treated with antibiotic combination therapy and with imipenem, respectively, septicemia was confirmed by positive blood cultures. Nineteen febrile episodes occurred in the myeloaplastic phase after bone marrow transplantation. In a comparative study of imipenem as monotherapy versus an antibiotic combination therapy the results obtained with imipenem were superior in many regards. No resistance developed necessitating change of antibiotic therapy. Coagulase-negative Staphylococci, primarily responsible for catheter-associated septicemia, were susceptible. Duration of fever and thus duration of treatment were shorter. The incidence of side effects and costs were lower. Therefore, imipenem as an antibiotic monotherapy in febrile cancer patients with neutropenia appears to be more efficacious than the conventional combination therapy, even during myeloaplasia following bone marrow transplantation. The results and rationale of this retrospective analysis are discussed.
Adolescent↗